Oncologic Imaging - ST Tumors

Heung Sik Kang
Sung Hwan Hong

Ja-Young Choi
Hye Jin Yoo
Oncologic Imaging
Soft Tissue Tumors
123
Oncologic Imaging: Soft Tissue Tumors
HeungSikKang SungHwanHong
Ja-YoungChoi HyeJinYoo
Oncologic Imaging:
Soft Tissue Tumors
HeungSikKang, MD SungHwanHong, MD
Department of Radiology Department of Radiology
Seoul National University College Seoul National University College
of Medicine of Medicine
Seoul National University Bundang Seoul National University Hospital
Hospital Seoul
Seongnam Korea, Republic of (South Korea)
Korea, Republic of (South Korea)
HyeJinYoo, MD
Ja-YoungChoi, MD Department of Radiology
Department of Radiology Seoul National University Hospital
Seoul National University Hospital Seoul
Seoul Korea, Republic of (South Korea)
Korea, Republic of (South Korea)
ISBN 978-981-287-717-8ISBN 978-981-287-718-5(eBook)

DOI 10.1007/978-981-287-718-5
Library of Congress Control Number: 2017940416
Springer Science+Business Media Singapore 2017

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The registered company address is: 152 Beach Road, #21-01/04 Gateway East, Singapore
189721, Singapore
To my coauthors, Sung Hwan Hong, Ja-Young Choi, and Hye
Jin Yoo, for their passion, excellence, and cooperation.
And to my three lovely grandsons, wishing them a bright and
prosperous future.
Heung Sik Kang, MD
To all the teachers and colleagues who have brought me to this

point.
And to my sweet wife, Yun Kyung, and daughter, Jisu, my
buttress and love.
Sung Hwan Hong, MD
To my colleagues and family.

And finally to my mentor, Professor Kang, for his new start.
Ja-Young Choi, MD
To my colleagues without whom I would not be here.

To my family for their endless support.
And to my cute daughter, whose smile warms my heart with
love.
Hye Jin Yoo, MD
Preface
Diagnostic imaging of soft tissue tumors remains a challenge in musculoskel-

etal radiology. The difficulty can be attributed to the diversity of soft tissue
tumors and the significant overlap in their imaging features. Moreover, con-
fusion persists in the literature regarding the terminology of soft tissue tumors
because their taxonomy has evolved over time.
Since the discovery of X-ray, radiologic examination has been widely used
to assess bone tumors, whereas soft tissue tumor evaluation was not broadly
possible until the introduction of magnetic resonance imaging (MRI) in the
early 1980s. Since then, remarkable progress has been made in the imaging
of soft tissue tumors. Today, MRI is indispensable in the management of
patients with soft tissue tumors, and its utility continues to increase as newly
developed technologies become clinically available. As a result, radiology
now plays a pivotal role in the detection, diagnosis, and treatment planning of
soft tissue tumors.
This book presents a wide range of radiologically illustrated soft tissue
tumors classified according to the 2013 WHO classification for soft tissue
tumor. Not only radiologists but also other specialists should become well
versed in the imaging evaluation of soft tissue tumors, to allow a multidisci-
plinary approach to treatment. As a radiology textbook, this book aims to
familiarize readers with both the imaging findings and pattern analysis of soft
tissue tumors. We hope that it will allow readers to improve their understand-
ing of soft tissue tumors and facilitate communication among physicians.
Seongnam, Korea, Republic of (South Korea) Heung Sik Kang

Seoul, Korea, Republic of (South Korea) Sung Hwan Hong
Seoul, Korea, Republic of (South Korea) Ja-Young Choi
Seoul, Korea, Republic of (South Korea) Hye Jin Yoo
vii
Contents
Part IGeneral Considerations for the Diagnosis of Soft

Tissue Tumors
1 Diagnostic Approach 3
1.1 Clinical Consideration 3
1.2 Imaging Modalities 4
1.2.1 Radiography 4
1.2.2 Ultrasonography 4
1.2.3 CT 4
1.2.4 MR Imaging 4
1.3 Benign Versus Malignant 5
1.4 Illustrations: Diagnostic Approach 6
1.4.1 Location ofSoft Tissue Tumors 6
1.4.2 Soft Tissue Tumor inClinical Syndrome 8
1.4.3 Radiographic Evaluation 9
1.4.4 Sonographic Evaluation 13
1.4.5 CT Evaluation 14
1.4.6 MR Imaging Evaluation 15
References 17
2 Diagnostic Procedure 19
2.1 Anatomic Compartments 19
2.1.1 Upper Extremity 19
2.1.2 Pelvis 19
2.1.3 Lower Extremity 20
2.2 Imaging-Guided Percutaneous Biopsy 20
2.3 Illustrations: Diagnostic Procedure 21
2.3.1 Anatomic Compartments 21
2.3.2 General Principles ofImage-Guided Percutaneous
Biopsy 24
References 26
3 Staging ofSoft Tissue Sarcoma 27
3.1 Tumor (T-Staging) 27
3.2 Nodes (N-Stage) 28
3.3 Metastasis (M-Stage) 29
3.4 Histologic Grade (G-Stage) 29
3.5 Proposed Report Format 29
ix
x Contents
3.6 Illustrations: Staging ofSoft Tissue Sarcoma 29

3.6.1 Stage I 29
3.6.2 Stage II 32
3.6.3 Stage III 33
3.6.4 Stage IV 35
References 36
Part IIWHO Classification of Soft Tissue Tumors and Radiologic

Illustrations
4 Adipocytic Tumors 39
4.1 Lipoma 39
4.2 Lipomatosis 40
4.3 Lipomatosis of Nerve 40
4.4 Lipoblastoma/Lipoblastomatosis 40
4.5 Angiolipoma 41
4.6 Spindle Cell Lipoma 41
4.7 Hibernoma 41
4.8 Lipoma Arborescens 42
4.9 Atypical Lipomatous Tumor/Well-Differentiated
Liposarcoma 42
4.10 Dedifferentiated Liposarcoma 42
4.11 Myxoid Liposarcoma 43
4.12 Pleomorphic Liposarcoma 44
4.13 Illustrations: Adipocytic Tumors 44
4.13.1 Lipoma 44
4.13.2 Lipomatosis 46
4.13.3 Lipomatosis of Nerve 47
4.13.4 Lipoblastoma 48
4.13.5 Angiolipoma 49
4.13.6 Spindle Cell Lipoma 50
4.13.7 Hibernoma 51
4.13.8 Lipoma Arborescens 53
4.13.9 Well-Differentiated Liposarcoma 54
4.13.10 Dedifferentiated Liposarcoma 56
4.13.11 Myxoid Liposarcoma 57
4.13.12 Pleomorphic Liposarcoma 60
References 61
5 Fibroblastic/Myofibroblastic Tumors 63
5.1 Nodular Fasciitis 63
5.2 Proliferative Fasciitis 63
5.3 Proliferative Myositis 64
5.4 Myositis Ossificans 64
5.5 Elastofibroma 65
5.6 Fibrous Hamartoma ofInfancy 65
5.7 Fibromatosis Colli 65
5.8 Fibroma ofTendon Sheath 66
5.9 Desmoplastic Fibroblastoma 66
Contents xi
5.10 Calcifying Aponeurotic Fibroma 66

5.11 Palmar/Plantar Fibromatosis 67
5.12 Desmoid-Type Fibromatosis 67
5.13 Dermatofibrosarcoma Protuberans 67
5.14 Solitary Fibrous Tumor 68
5.15 Inflammatory Myofibroblastic Tumor 68
5.16 Myxofibrosarcoma 69
5.17 Low-Grade Fibromyxoid Sarcoma 69
5.18 Low-Grade Myofibroblastic Sarcoma 69
5.19 Sclerosing Epithelioid Fibrosarcoma 69
5.20 Illustrations: Fibroblastic/Myofibroblastic Tumors 70
5.20.1 Nodular Fasciitis 70
5.20.2 Proliferative Fasciitis 73
5.20.3 Proliferative Myositis 74
5.20.4 Myositis Ossificans 76
5.20.5 Elastofibroma Dorsi 80
5.20.6 Fibrous Hamartoma ofInfancy 81
5.20.7 Fibromatosis Colli 83
5.20.8 Fibroma ofTendon Sheath 84
5.20.9 Desmoplastic Fibroblastoma 87
5.20.10 Calcifying Aponeurotic Fibroma 89
5.20.11 Palmar/Plantar Fibromatosis 90
5.20.12 Desmoid-Type Fibromatosis 91
5.20.13 Dermatofibrosarcoma Protuberans 94
5.20.14 Solitary Fibrous Tumor 96
5.20.15 Inflammatory Myofibroblastic Tumor 98
5.20.16 Myxofibrosarcoma 99
5.20.17 Low-Grade Fibromyxoid Sarcoma 101
5.20.18 Low-Grade Myofibroblastic Sarcoma 104
5.20.19 Sclerosing Epithelioid Fibrosarcoma 106
References 106
6 So-Called Fibrohistiocytic Tumors 109
6.1 Tenosynovial Giant Cell Tumor 109
6.2 Deep Benign Fibrous Histiocytoma 110
6.3 Giant Cell Tumor ofSoft Tissue 110
6.4 Illustrations: So-Called Fibrohistiocytic Tumors 111
6.4.1 Tenosynovial Giant Cell Tumor, Localized Type 111
6.4.2 Tenosynovial Giant Cell Tumor, Diffuse Type 115
6.4.3 Giant Cell Tumor ofSoft Tissue 118
6.4.4 Deep Benign Fibrous Histiocytoma 119
References 120
7 Smooth Muscle Tumors 121
7.1 Leiomyoma ofDeep Soft Tissue 121
7.2 Leiomyosarcoma 121
7.3 Illustrations: Smooth Muscle Tumors 122
7.3.1 Leiomyoma ofDeep Soft Tissue 122
7.3.2 Leiomyosarcoma 126
References 129
xii Contents
8 Pericytic (Perivascular) Tumors 131

8.1 Glomus Tumor 131
8.2 Myopericytoma, Including Myofibroma 132
8.3 Angioleiomyoma 132
8.4 Illustrations: Pericytic (Perivascular) Tumors 133
8.4.1 Glomus Tumor 133
8.4.2 Glomangioma 137
8.4.3 Glomangiomatosis 138
8.4.4 Myofibroma 139
8.4.5 Myopericytoma 142
8.4.6 Angioleiomyoma 143
References 147
9 Skeletal Muscle Tumors 149
9.1 Rhabdomyosarcoma 149
9.2 Illustrations: Skeletal Muscle Tumors 150
9.2.1 Embryonal Rhabdomyosarcoma 150
9.2.2 Alveolar Rhabdomyosarcoma 151
9.2.3 Pleomorphic Rhabdomyosarcoma 152
9.2.4 Spindle Cell Rhabdomyosarcoma 155
References 156
10 Vascular Tumors 157
10.1 Hemangiomas 157
10.2 Angiomatosis 158
10.3 Lymphangioma 159
10.4 Kaposiform Hemangioendothelioma 159
10.5 Retiform Hemangioendothelioma 159
10.6 Kaposi Sarcoma 159
10.7 Epithelioid Hemangioendothelioma 160
10.8 Angiosarcoma ofSoft Tissue 160
10.9 Illustrations: Vascular Tumors 161
10.9.1 Hemangioma 161
10.9.2 Synovial Hemangioma 162
10.9.3 Intramuscular Angioma 164
10.9.4 Ossifying Intramuscular Angioma 168
10.9.5 Venous Hemangioma 169
10.9.6 Arteriovenous Malformation/Hemangioma 171
10.9.7 Angiomatosis 172
10.9.8 Lymphangioma 173
10.9.9 Kaposiform Hemangioendothelioma 175
10.9.10 Retiform Hemangioendothelioma 177
10.9.11 Kaposi Sarcoma 178
10.9.12 Epithelioid Hemangioendothelioma 179
10.9.13 Angiosarcoma ofSoft Tissue 182
References 184
11 Chondro-Osseous Tumors 185
11.1 Soft Tissue Chondroma 185
11.2 Extraskeletal Osteosarcoma 185
11.3 Illustrations: Chondro-Osseous Tumors 186
Contents xiii
11.3.1 Soft Tissue Chondroma 186

11.3.2 Extraskeletal Osteosarcoma 190
References 191
12 Nerve Sheath Tumors 193
12.1 Schwannoma (Including Variants) 193
12.2 Neurofibroma (Including Variants) 194
12.3 Perineurioma 195
12.4 Granular Cell Tumor 195
12.5 Malignant Peripheral Nerve Sheath Tumor 196
12.6 Illustrations: Nerve Sheath Tumors 197
12.6.1 Schwannoma 197
12.6.2 Cellular Schwannoma 201
12.6.3 Plexiform Schwannoma 202
12.6.4 Ancient Schwannoma 204
12.6.5 Neurofibroma 207
12.6.6 Extraneural Perineurioma 211
12.6.7 Intraneural Perineurioma 212
12.6.8 Granular Cell Tumor 213
12.6.9 Malignant Granular Cell Tumor 215
12.6.10 Malignant Peripheral Nerve Sheath Tumor 216
References 220
13 Tumors ofUncertain Differentiation 221
13.1 Intramuscular Myxoma 221
13.2 Synovial Sarcoma 222
13.3 Phosphaturic Mesenchymal Tumor 222
13.4 Epithelioid Sarcoma 223
13.5 Alveolar Soft Part Sarcoma 224
13.6 Clear Cell Sarcoma 224
13.7 Extraskeletal Myxoid Chondrosarcoma 225
13.8 Extraskeletal Ewing Sarcoma 225
13.9 Extrarenal Malignant Rhabdoid Tumor 226
13.10 Myoepithelioma/Parachordoma 226
13.11 Pleomorphic Hyalinizing Angiectatic Tumor 226
13.12 Illustrations: Tumors ofUncertain Differentiation 227
13.12.1 Intramuscular Myxoma 227
13.12.2 Synovial Sarcoma 230
13.12.3 Phosphaturic Mesenchymal Tumor 234
13.12.4 Epithelioid Sarcoma 236
13.12.5 Angiosarcoma 239
13.12.6 Clear Cell Sarcoma 241
13.12.7 Extraskeletal Myxoid Chondrosarcoma 243
13.12.8 Extraskeletal Ewing Sarcoma 245
13.12.9 Extrarenal Rhabdoid Tumor 247
13.12.10 Myoepithelioma 248
13.12.11 Pleomorphic Hyalinizing Angiectatic Tumor
ofSoft Part 249
References 250
xiv Contents
14 Undifferentiated/Unclassified Sarcoma 253

14.1 Undifferentiated Pleomorphic Sarcoma 253
14.2 Undifferentiated RoundCell andSpindle Cell Sarcoma 254
14.3 Illustrations: Undifferentiated/Unclassified Sarcoma 255
14.3.1 Undifferentiated Pleomorphic Sarcoma 255
14.3.2 CIC-DUX4 Fusion Positive Undifferentiated
RoundCell Sarcoma 261
References 262
15 Superficial Soft Tissue Masses 263
15.1 Epidermal Inclusion Cyst 263
15.2 Pilomatricoma 264
15.3 Fat Necrosis 264
15.4 Rheumatoid Nodule 265
15.5 Morel-Lavallee Lesion 265
15.6 Malignant Melanoma 266
15.7 Lymphoma (Cutaneous andSubcutaneous) 266
15.8 Soft Tissue Metastasis 268
15.9 Illustrations: Superficial Soft Tissue Masses 269
15.9.1 Epidermal Inclusion Cyst 269
15.9.2 Pilomatricoma 272
15.9.3 Fat Necrosis 275
15.9.4 Rheumatoid Nodule 276
15.9.5 Morel-Lavallee Lesion 278
15.9.6 Malignant Melanoma 279
15.9.7 Lymphoma (Cutaneous andSubcutaneous) 283
15.9.8 Soft Tissue Metastasis 286
References 287
16 Masses That May Mimic Soft Tissue Tumors 291
16.1 Ganglion 291
16.2 Vascular Lesion 291
16.3 Gout 292
16.4 Sarcoidosis 293
16.5 Mortons Neuroma 293
16.6 Traumatic Neuroma 294
16.7 Xanthoma 295
16.8 Illustrations: Masses That May Mimic Soft Tissue
Tumors 296
16.8.1 Ganglion 296
16.8.2 Vascular Lesion 299
16.8.3 Gout 301
16.8.4 Sarcoidosis 303
16.8.5 Mortons Neuroma 306
16.8.6 Traumatic Neuroma 308
16.8.7 Xanthoma 311
References 314
Contents xv
Part III Practical Pearls in Diagnosis of Soft Tissue Tumors

17 Lesion Characterization Based onMR Signal Intensities 317
17.1 T1 Hyperintense Lesions 319
17.1.1 Lesion Containing Fat 319
17.1.2 Lesion Containing Methemoglobin 322
17.1.3 Lesion Containing Proteinaceous Material 323
17.1.4 Lesion Containing Melanin 324
17.2 T2 Hypointense Lesions 325
17.2.1 Lesion Containing Fibrous Tissue 325
17.2.2 Lesion Containing Mineralization 329
17.2.3 Lesion Containing Free Cholesterol 334
17.2.4 Lesion Containing High-Flow Vessels 334
17.3 T2 Fluid-Equivalent Hyperintense Lesions 337
17.3.1 Lesion Containing Fluid 337
17.3.2 Lesion Containing Myxoid Tissue 339
17.3.3 Vascular Lesion 342
17.3.4 Lesions Containing Cartilage 343
18 Diagnostic Signs 345
18.1 Bowl ofGrapes Sign 345
18.2 Bunch ofGrapes Appearance 346
18.3 Checkerboard Appearance 347
18.4 Coaxial Cable-Like Appearance 348
18.5 Dark Star Sign 349
18.6 Fascicular Sign 350
18.7 Inverted Target Sign 351
18.8 Gyriform Pattern 352
18.9 Reverse Target Sign 353
18.10 Reverse Zoning Phenomenon 354
18.11 Spaghetti-Like Appearance 355
18.12 Split Fat Sign 356
18.13 String Sign 357
18.14 Stripe Sign 358
18.15 Swiss Cheese Appearance 359
18.16 Tail Sign 360
18.17 Target Sign 361
18.18 Three Stripes Sign 362
18.19 Triple Signal Sign 363
18.20 Zonal Phenomenon 364
19 Syndrome-Associated Soft Tissue Tumors 365
19.1 Kasabach-Merritt Syndrome 366
19.2 Maffucci Syndrome 368
19.3 Klippel-Trenaunay Syndrome 370
19.4 Mazabraud Syndrome 371
19.5 Neurofibromatosis Type 1 372
xvi Contents

19.7 Schwannomatosis 379
19.8 Carney Complex 380
19.9 Familial Hypercholesterolemia 382
Part IV Drill and Practice
20 Image Interpretation Session 387

20.1 Quiz 388
20.2 Quiz 390
20.3 Quiz 392
20.4 Quiz 394
20.5 Quiz 396
20.6 Quiz 398
20.7 Quiz 400
20.8 Quiz 402
20.9 Quiz 404
20.10 Quiz 406
20.11 Quiz 408
20.12 Quiz 410
20.13 Quiz 412
20.14 Quiz 414
20.15 Quiz 416
20.16 Quiz 418
20.17 Quiz 420
20.18 Quiz 422
20.19 Quiz 424
20.20 Quiz 426
20.21 Quiz 428
20.22 Quiz 430
20.23 Quiz 432
20.24 Quiz 434
20.25 Quiz 436
20.26 Quiz 438
20.27 Quiz 440
20.28 Quiz 442
20.29 Quiz 444
20.30 Quiz 446
20.31 Quiz 448
20.32 Quiz 450
20.33 Quiz 452
Part I
General Considerations for the Diagnosis
of Soft Tissue Tumors
Diagnostic Approach
1
1.1 Clinical Consideration important for limiting the differential diagnosis

(Walker etal. 2011a). For example, the major-
The vast majority of soft tissue masses that ity of soft tissue myxomas are intramuscular,
present to physicians are benign lesions. The whereas myxoid liposarcomas are usually inter-
incidence of benign soft tissue tumors is esti- muscular lesions (Murphey etal. 2002). Certain
mated to outnumber malignant tumors by a fac- tumors are known for their inherent predilec-
tor of at least 100 (Balach etal. 2011). Unlike tions for specific locations, such as elastofi-
their intraosseous counterparts, it is often not broma, which is typically found in the
possible to establish a meaningful differential infrascapular or subscapular area. In a similar
diagnosis for soft tissue tumors or reliably fashion, recognizing that a lesion arises from a
determine if they are benign or malignant. In specific structure (e.g., nerves, vessels, or ten-
these cases, knowledge of the soft tissue dons) can help in lesion characterization (Wu
tumors prevalence, along with the patients age and Hochman 2009). A multifocal or an exten-
and the lesions location, will allow a suitably sive lesion also limits diagnostic considerations
ordered differential diagnosis (Kransdorf to include angiomatous lesions, neurofibroma-
1995). Embryonal rhabdomyosarcoma occurs tosis (NF), fibromatosis, lipomatosis, myxoma
most often in children under 10years of age, (Mazabraud syndrome), metastases, or lym-
while synovial sarcoma is most prevalent in phoma (Walker etal. 2011b).
adolescents and young adults. Most soft tissue Clinical history may be relevant in systemic
sarcomas, including undifferentiated pleomor- or concomitant diseases, such as melanotic
phic sarcoma, liposarcoma, and leiomyosar- schwannoma in Carney syndrome (cardiac
coma, dominate in the elderly. The most myxoma, spotty pigmentation, and endocrine
common tumors in childhood and adolescence overactivity), cavernous hemangioma in
are, in order of prevalence, hemangioma, Maffucci syndrome (enchondromatosis), fibro-
fibrous hamartoma, granuloma annulare, lipomatosis in Gardner syndrome (intestinal pol-
blastoma, fibrosarcoma, and rhabdomyosar- yposis, osteomata), xanthoma in familial
coma. The most common tumors in adults are hypercholesterolemia, myxoma in Mazabraud
lipoma, liposarcoma, and myxofibrosarcoma syndrome (polyostotic fibrous dysplasia), amy-
(De Schepper and Bloem 2007). loidoma in multiple myeloma and neurofibro-
Lesion locations include subcutaneous, peri- mata, and schwannomata in neurofibromatosis
fascial, intramuscular, intermuscular, and intra- type II and schwannomatosis (De Schepper and
articular/periarticular. The lesion location is Bloem 2007).
Springer Science+Business Media Singapore 2017 3

H.S. Kang et al., Oncologic Imaging: Soft Tissue Tumors, DOI10.1007/978-981-287-718-5_1
4 1 Diagnostic Approach
1.2 Imaging Modalities nonspecific (Hwang and Panicek 2009). US is in

most instances the primary imaging modality in
1.2.1 Radiography the evaluation of soft tissue masses in children and
is particularly useful in the diagnosis of vascular
Although the utility of radiographs in evaluating lesions (Navarro 2011). US is the most popular
soft tissue lesions is rather limited, some radio- imaging modality for biopsies of soft tissue
graphic findings help to detect or characterize tumors. Sonographic guidance is suitable for tar-
soft tissue lesions. At radiography, lipoma or geting viable tissues, avoiding necrotic portions in
well-differentiated liposarcoma can be appreci- soft issue tumors, and obviating the risk of neuro-
ated as a low-density mass. If radiography shows vascular injury.
a soft tissue mass with multiple phleboliths, it is
most likely hemangioma. A soft tissue mass with
a central amorphous bone formation is suggestive 1.2.3 CT
of extraskeletal osteosarcoma, whereas a soft tis-
sue lesion with peripheral shell-like mineraliza- Although MR imaging is preferred for evaluating
tion is characteristic of myositis ossificans. The soft tissue tumor imaging, there are distinguish-
presence of multiple punctate or curvilinear cal- ing CT characteristics that can suggest a specific
cifications is indicative of an extraskeletal chon- diagnosis, including the lesions mineralization
droid tumor. Other examples of intratumoral pattern, density, pattern of adjacent bone involve-
mineralization include pilomatricoma, calcifying ment, and degree and pattern of vascularity
aponeurotic fibroma, gouty tophus, and synovial (Subhawong etal. 2010). CT remains superior to
sarcoma. Localized cortical thickening or hyper- radiography and MR imaging in demonstrating
ostosis may occur adjacent to soft tissue heman- soft tissue mineralization, and the mineralization
giomas or lipomas (Ly etal. 2003; Kim etal. pattern of soft tissue masses can be a clue for the
1999). Any tumor involvement with the adjacent histologic diagnosis. In myositis ossificans, for
bone may be better evaluated with radiography example, CT is more sensitive than MR imaging
than with MR imaging. For example, long-term in detecting early-stage mineralization with zonal
pressure erosion may be easily distinguished phenomenon. Lesion density can also suggest a
from an aggressive permeative destructive pat- histologic diagnosis, particularly in soft tissue
tern on radiography, whereas it may be confusing masses containing fat density. CT is excellent in
on MR imaging (Manaster 2013). delineating the presence and pattern of bone
involvement in soft tissue tumors, which are
important factors during preoperative planning.
1.2.2 Ultrasonography CT has also become essential in radiation ther-
apy, and the use of CT has resulted in greater pre-
Because of its safety, availability, feasibility, and cision in dose distribution, patient positioning,
cost-effectiveness, ultrasonography (US) is fre- and 3D dose calculation (Pereira etal. 2014).
quently used as the initial imaging technique in the
evaluation of soft tissue masses. One of the pri-
mary roles of US is to determine whether a mass is 1.2.4 MR Imaging
a cyst or a solid tumor. Superficial soft tissue
masses are particularly amenable to high- MR imaging is the diagnostic modality of choice
resolution US, which allows a detailed assessment for soft tissue tumors due to the high tissue con-
of the distinguishing ultrasound characteristics that trast and multiplanar capability. MR imaging is
enable a specific tumor diagnosis based on recog- well suited for not only the diagnosis but also for
nized ultrasound appearances (Hung etal. 2014). the staging, preoperative planning, postsurgical
Malignant tumors are usually hypoechoic and often evaluation, and post-therapy surveillance of soft
are hypervascular, but the sonographic appearances tissue tumors. MR images can be particularly
of solid soft tissue tumors are otherwise usually useful for characterizing benign lesions that do
1.3 Benign Versus Malignant 5
not require imaging follow-up or biopsy, such as 1.3 Benign Versus Malignant
lipomas and ganglia. In cases where a soft tissue
lesion is indeterminate based on the clinical and MR imaging provides clear advantages in terms of
imaging features, a biopsy should be considered differential diagnosis of soft tissue tumors. There
(Wu and Hochman 2009). is, however, much controversy regarding the value
A combination of T1- and T2-weighted images of MR imaging in the differentiation of benign and
is the mainstay of MR imaging of soft tissue malignant soft tissue tumors. MR imaging differ-
tumors. Fat-suppression techniques are widely entiation between benign and malignant tumors is
adopted to enhance the dynamic ranges and sensi- complicated by the low prevalence of these lesions,
tivity of fast spin echo T2-weighted images and the minimal experience of radiologists in non-ded-
gadolinium-enhanced T1-weighted images. A icated hospitals, the ambiguous information on
T2*-weighted gradient-echo sequence is helpful MR signal intensities, the highly variable histo-
in the detection of blood products, such as hemo- logical findings, and the natural evolution of the
siderin. The administration of intravenous gado- lesions (Garcia-Gomez etal. 2004).
linium chelates is used to distinguish cystic from The definite malignant indicators are distant
solid components to identify viable and necrotic metastasis and adjacent organ invasion. The like-
areas, to show the relative vascularity of tumors, lihood of malignancy also increases with the
and to delineate the true margin of tumors. presence of tumor necrosis, neurovascular
Most soft tissue tumors are T1 isointense or encasement, and bone invasion. The integrity of
hypointense and T2 hyperintense in signal inten- the deep fascia may also be a differential factor.
sity. The presence of T1 hyperintensity or T2 The trend toward invasive behavior is greater in
hypointensity in soft tissue tumors is occasion- malignant than in benign tumors, and the destruc-
ally found and helpful in differential diagnosis tion of the deep fascia on MR imaging can be a
when present. An intratumoral T1 hyperintensity useful imaging finding in identifying malignant
is suggestive of fat, subacute hemorrhage, high tumors (Liu etal. 2011).
proteinaceous fluid, or melanin. Fat-suppression Although benign tumors tend to be well delin-
techniques are helpful to distinguish fat from the eated and some malignant tumors have ill-defined
other T1-hyperintense materials. T2-hypointense margins, several studies have concluded that the
substances include calcifications/ossifications, margin of a soft tissue mass on MR imaging is of
hemorrhages, vascular signal voids, or collage- no statistical relevance in predicting malignancy
nous tissues. The T2-hypointense element can be (De Schepper etal. 2000). In fact, most soft tis-
a clue of some benign soft tissue tumors such as sue tumors have well-defined margins regardless
tenosynovial giant cell tumor, fibromatosis, and of whether they are benign or malignant. The
desmoplastic fibroblastoma. However, various administration of a contrast agent provides fur-
soft tissue sarcomas may also have T2-hypointense ther information on the MR imaging characteris-
components. Fluid-containing lesions exhibit tics of soft tissue tumors; however, it does not
very high signal intensity on T2-weighted permit the discrimination between benign and
images, which allows the specific diagnosis of malignant lesions when evaluated qualitatively.
cystic masses, such as ganglion or bursitis. Dynamic contrast enhancement MR imaging can
Myxoid tumors also exhibit very high signal on be used to differentiate malignant from benign
T2-weighted images because of their high water soft tissue tumors (Tuncbilek etal. 2005).
content. Contrast enhancement is helpful to dif- In superficial soft tissue tumors, which are
ferentiate these bright T2-weighted signal inten- defined as masses located within the subcutaneous
sity lesions. Contrast-enhanced MR images also layer, the following various imaging features are
show the vascularity of soft tissue tumors. known to be related to malignancy: lobulation,
Although malignant lesions are apt to show rapid hemorrhage, necrosis, fascial edema, skin thicken-
and greater enhancement, the presence of con- ing, and skin contact. However, size was not found
trast enhancement does not distinguish benign to be an important determining factor for malig-
from malignant tumors. nancy, with a significant proportion of malignant
superficial sarcomas measuring less than 5cm in ses and a mean diameter greater than 66mm (De
maximal diameter (Calleja etal. 2012). Schepper etal. 1992).
A study to assess the accuracy of MR imaging A simplified systemic MR imaging approach
in predicting malignancy revealed that the has been proposed to help predict the benign or
absence of low signal intensity on T2-weighted malignant nature of soft tissue tumors (Chung
images, a mean diameter greater than 33mm, and etal. 2012). The combination of the following
a heterogeneous signal on T1-weighted images three parameters arranged in order resulted in a
were the most sensitive indicators of malignant higher diagnostic value for malignancy: signal
soft tissue tumors. MR imaging features with the intensity (heterogeneity on T2-weighted images),
highest specificity were evidence of necrosis, size (50mm), and depth (deep relative to the
bone or neurovascular involvement, or metasta- superficial investing fascia).
1.4 Illustrations: Diagnostic Approach
1.4.1 Location ofSoft Tissue Tumors
a b
c d
Fig. 1.1 Location of soft tissue tumors. Axial T2WIs show subcutaneous (a), perifascial (b), subfascial (c), and intra-
muscular (d) masses
a b
Fig. 1.2 Intra-versus intermuscular location. Axial T2WI (a) shows an intramuscular myxoma in the left vastus media-
lis. Axial FS PDWI (b) shows an intermuscular mass proven to be a myxoid liposarcoma in the left thigh
Fig. 1.3 Elastofibroma dorsi. There are bilateral sym-

metrical soft tissue masses (arrows) in the chest wall
between the rib cage and serratus anterior. This tumor
almost always arises in this very specific location
1.4.2 S
oft Tissue Tumor inClinical
Syndrome
Fig. 1.4 Soft tissue
hemangioma in
Maffucci syndrome.
AP radiograph of both
hands shows multiple
enchondromas in the
left hand and wrist.
There is a soft tissue
hemangioma (arrow)
with multiple calcific
foci and adjacent bone
erosions on the radial
side of the right wrist
1.4.3 Radiographic Evaluation
a b
Fig. 1.5 Well-differentiated liposarcoma. AP radiograph shows a large fatty mass on the medial side of the right
of the right knee (a) shows a large soft tissue mass with distal thigh
low density indicating fat component. Coronal T1WI (b)
a b
Fig. 1.6 Hemangioma. AP radiograph of the left elbow (a) shows a large soft tissue mass with numerous phleboliths. Sagittal
T2WI (b) shows a complex and infiltrative soft tissue mass with multiple fluid-fluid levels on the left anterior elbow
a b
Fig. 1.7Myositis
ossificans. AP (a) and
lateral (b) radiographs
of the right knee show a
mineralized soft tissue
mass (arrow) on the
posteromedial side of
the right distal femur.
The mineralization
pattern is consistent
with myositis
ossificans, which
exhibits a characteristic
zonal phenomenon
a b
Fig. 1.8 Synovial sarcoma. AP oblique radiograph (a) T1WI (b) shows a poorly demarcated enhancing soft tis-
shows a nonspecific calcification (arrow) on the lateral sue mass with a central low signal calcification (arrow),
side of the left mid lower leg. Coronal postcontrast FS one of the well-known features of synovial sarcoma
Fig. 1.9Cortical
hyperostosis adjacent to a b
soft tissue hemangioma.
AP radiograph of the
right thigh (a) shows a
segmental cortical
thickening (arrow) on
the lateral side of the
right femur proximal
diaphysis. Coronal FS
T2WI (b) shows an
extensive soft tissue
hemangioma with
cortical hyperostosis
(arrow) in the right
proximal femur
1.4.4 Sonographic Evaluation
a b
Fig. 1.10 Myxoid liposarcoma. Axial T2WI (a) shows a (c) show complex internal echogenicity and intratumoral
subfascial mass (arrow) of the right sartorius. The mass vascularity, indicating myxoid tissue rather than fluid
has a very high signal, which may suggest a cystic or content
myxoid nature. Transverse US (b) and power Doppler US
1.4.5 CT Evaluation
a b
Fig. 1.11 Ossifying hemangioma. AP radiograph (a) images (b, c) clearly depict the extent of cortical change
shows cortical hyperostosis (arrow) of the left femur and (arrow) and the internal architecture of the ossified mass
a juxtacortical ossified mass. Coronal and axial CT
1.4.6 MR Imaging Evaluation
a b
c d
Fig. 1.12 Rhabdomyosarcoma. Axial postcontrast FS trast FS T1WI at postoperative 3months (c) shows post-
T1WI (a) shows a rhabdomyosarcoma in the anterior surgical scar with no residual or recurred tumors. Axial
compartment of the right forearm. Axial postcontrast FS postcontrast T1WI at postoperative 14months (d) reveals
T1WI after neoadjuvant chemotherapy (b) shows a a metastatic mass on the medial side of the right upper
marked decrease in the size of the mass. Axial postcon- arm
a b
c d
Fig. 1.13 MR predictors of malignant soft tissue tumor. vial sarcoma), and bone invasion (arrow) (d, undifferenti-
Axial MR images show heterogeneity on T2WI (a, malig- ated pleomorphic sarcoma). All of these aggressive
nant peripheral nerve sheath tumor), necrotic changes imaging features favor a diagnosis of malignant soft tissue
(arrows) on postcontrast FS T1WI (b, extraskeletal Ewing tumor
sarcoma), popliteal artery encasement (arrow) (c, syno-
References 17
a b
Fig. 1.14 Dynamic contrast-enhanced MRI.Axial post- upper arm. Dynamic contrast-enhanced MRI (b) shows
contrast FS T1WI (a) shows a subfascial soft tissue mass rapid early enhancement followed by washout enhance-
with nonspecific heterogeneous enhancement in the right ment pattern which is predictive of malignancy
Garcia-Gomez JM, Vidal C, Marti-Bonmati L, Galant J,

References Sans N, Robles M, Casacuberta F.Benign/malignant
classifier of soft tissue tumors using MR imaging.
Balach T, Stacy GS, Haydon RC.The clinical evaluation MAGMA. 2004;16(4):194201. doi:10.1007/
of soft tissue tumors. Radiol Clin N Am. s10334-003-0023-7.
2011;49(6):118596. doi:10.1016/j.rcl.2011.07.005. Hung EHY, Griffith JF, Hung Ng AW, Lee RKL, Lau
Calleja M, Dimigen M, Saifuddin A.MRI of superficial DTY, Leung JCS.Ultrasound of musculoskeletal soft-
soft tissue masses: analysis of features useful in distin- tissue tumors superficial to the investing fascia. Am
guishing between benign and malignant lesions. JRoentgenol. 2014;202(6):W53240. doi:10.2214/
Skelet Radiol. 2012;41(12):151724. doi:10.1007/ AJR.13.11457.
s00256-012-1385-6. Hwang S, Panicek DM.The evolution of musculoskeletal
Chung WJ, Chung HW, Shin MJ, Lee SH, Lee MH, Lee tumor imaging. Radiol Clin N Am. 2009;47(3):435
JS, Kim MJ, Lee WK.MRI to differentiate benign 53. doi:10.1016/j.rcl.2008.12.002.
from malignant soft-tissue tumours of the extremities: Kim JY, Jung SL, Park YH, Park SH, Kang YK.Parosteal
a simplified systematic imaging approach using depth, lipoma with hyperostosis. Eur Radiol. 1999;9(9):1810
size and heterogeneity of signal intensity. Br JRadiol. 2. doi:10.1007/s003300050927.
2012;85(1018):e8316. doi:10.1259/bjr/27487871. Kransdorf MJ.Benign soft-tissue tumors in a large refer-
De Schepper AM, Bloem JL.Soft tissue tumors: grading, ral population: distribution of specific diagnoses by
staging, and tissue-specific diagnosis. Top Magn age, sex, and location. AJR Am JRoentgenol.
Reson Imaging. 2007;18(6):43144. doi:10.1097/ 1995;164(2):395402. doi:10.2214/ajr.164.2.7839977.
rmr.0b013e3181652220. Liu L, Wu N, Ouyang H.Diagnostic value of delineating
De Schepper AM, De Beuckeleer L, Vandevenne J, deep fascia in distinguishing between benign and
Somville J.Magnetic resonance imaging of soft tissue malignant soft-tissue tumors in lower limbs using 3.0
tumors. Eur Radiol. 2000;10(2):21323. doi:10.1007/ T magnetic resonance imaging. JMagn Reson
s003300050037. Imaging. 2011;33(1):1739. doi:10.1002/jmri.22371.
De Schepper AM, Ramon FA, Degryse HR.Statistical Ly JQ, Sanders TG, Mulloy JP, Soares GM, Beall DP,
analysis of MRI parameters predicting malignancy in Parsons TW, Slabaugh MA.Osseous change adjacent
141 soft tissue masses. RoFo. 1992;156(6):58791. to soft-tissue hemangiomas of the extremities:
doi:10.1055/s-2008-1032948. correlation with lesion size and proximity to bone.
AJR Am JRoentgenol. 2003;180(6):1695700. tions: what does CT add to diagnosis and manage-
doi:10.2214/ajr.180.6.1801695. ment? AJR Am JRoentgenol. 2010;194(6):155967.
Manaster BJ.Soft-tissue masses: optimal imaging proto- doi:10.2214/AJR.09.3736.
col and reporting. AJR Am JRoentgenol. Tuncbilek N, Karakas HM, Okten OO.Dynamic contrast
2013;201(3):50514. doi:10.2214/AJR.13.10660. enhanced MRI in the differential diagnosis of soft
Murphey MD, McRae GA, Fanburg-Smith JC, Temple tissue tumors. Eur JRadiol. 2005;53(3):5005.
HT, Levine AM, Aboulafia AJ.Imaging of soft-tissue doi:10.1016/j.ejrad.2004.04.012.
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son of radiologic and pathologic findings. Radiology. MD.Magnetic resonance imaging of benign soft tissue
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Navarro OM.Soft tissue masses in children. Radiol Clin N 2011a;49(6):1197217. doi:10.1016/j.rcl.2011.07.007.
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Diagnostic Procedure
2
2.1 Anatomic Compartments coracobrachialis, and brachioradialis muscles.

The posterior compartment contains the triceps
Compartmental spaces are confined anatomical muscles.
areas that are separated by the fascia, interosse-
ous ligaments, or bones (Bancroft etal. 2007). 2.1.1.3 Forearm
These separate compartments may act as natural The forearm is divided into three compartments
barriers to tumor spread and limit the stage of a as follows: dorsal, volar, and mobile wad. The
malignancy (Anderson etal. 1999; Bancroft etal. dorsal compartment contains the extensor mus-
2007). The tissues that can provide barriers cles. The volar compartment contains the flexor
include the synovium, capsule, articular cartilage muscles. The interosseous membrane separates
of joints, cortical bone, periosteum, major fascial the two compartments. The mobile wad com-
septa, tendinous origins, and insertions of mus- prises the extensor carpi radialis brevis, longus,
cles. In contrast, the fat, areolar tissue, and mus- and brachioradialis muscles.
cle are relatively poor barriers to tumor spread,
and certain regions that are composed of these 2.1.1.4 Hand
tissues are considered completely extracompart- There are multiple compact compartments in the
mental (Anderson etal. 1999). hand and, therefore, several neurovascular bun-
dles. For this reason, most lesions involving the
hand region are considered extracompartmental.
2.1.1 Upper Extremity
2.1.1.5 Extracompartmental Spaces
2.1.1.1 Periscapular Region Extracompartmental spaces of the upper extrem-
The muscles and fasciae that cover the dorsal ity include the periclavicular region, axilla, ante-
scapula are considered a compartment. They cubital fossa, wrist, and dorsum of the hand.
include the infraspinatus, teres minor, and rhom-
boid muscles; the supraspinatus muscle is in a
separate compartment. 2.1.2 Pelvis
2.1.1.2 Upper Arm Individual bones or muscles in the pelvis are con-
There are two compartments in the upper arm as sidered individual compartments. However, extra-
follows: anterior and posterior. The anterior compartmental spread to adjacent tissues is
compartment contains the biceps, brachialis, frequently observed when pelvic lesions are large.

20 2 Diagnostic Procedure
2.1.3 Lower Extremity 2.1.3.4 Extracompartmental Spaces

The inguinal region, femoral triangle, popliteal
2.1.3.1 Thigh fossa, ankle, and dorsum of the foot are consid-
There are three compartments in the thigh as fol- ered extracompartmental.
lows: anterior, posterior, and medial. The anterior
compartment contains the iliotibial tract, tensor
lata, rectus femoris, and vastus muscles. The vas- 2.2 Imaging-Guided
tus intermedius muscle is occasionally consid- Percutaneous Biopsy
ered a separate fourth compartment. The posterior
compartment contains the hamstring muscles Percutaneous core needle biopsies of the muscu-
(semimembranosus, semitendinosus, and biceps loskeletal lesions are accurate and effective pro-
femoris) and the sciatic nerve. The medial com- cedures and carry the advantages of decreased
partment contains the gracilis and adductor complications and cost compared with open
muscles. biopsies (UyBico etal. 2012). However, there is
a concern that tumor seeding and recurrence can
2.1.3.2 Lower Leg occur along the needle track of percutaneous core
The lower leg contains four compartments as needle biopsy because survival rates for patients
follows: anterior, deep posterior, superficial who experience local recurrence of sarcomas are
posterior, and lateral. The anterior compartment very low. If a percutaneous biopsy passes through
contains the tibialis anterior, extensor digitorum tissues outside the planned incision plane, the
longus, and extensor hallucis longus muscles, as orthopedic oncologic surgeon may alter the sur-
well as the anterior tibial artery, vein, and deep gical plane to include the potentially contami-
peroneal nerve. The anterior compartment is nated tissue in the resection, which would require
separated from the deep posterior compartment a wider irradiation field or increase the chance of
by the interosseous membrane. The deep poste- local tumor recurrence at the biopsy site after the
rior compartment contains the tibialis posterior, operation (Liu etal. 2007). A multicenter study
flexor digitorum, and flexor hallucis longus conducted by the Musculoskeletal Tumor Society
muscles, with the posterior tibial and peroneal in 1996 showed that inappropriately performed
arteries and the tibial nerve. The superficial pos- biopsies of sarcomas led to unnecessary amputa-
terior compartment contains the gastrocnemius tions in 58% of patients (Mankin etal. 1996).
and soleus muscles and the sural nerve. The lat- Therefore, it is strongly suggested that the biopsy
eral compartment contains the peroneal mus- of soft tissue sarcomas be performed along the
cles, with the common and superficial peroneal plane of the planned incision for the definitive
nerves. resection surgery, thereby avoiding needle pene-
tration and contamination of uninvolved myofas-
2.1.3.3 Foot cial compartments (Liu etal. 2007). In general,
The dorsum of the foot is considered extracom- biopsies of primary musculoskeletal sarcomas
partmental. The sole of the foot is divided into should be performed only at institutions with a
three compartments as follows: medial, central, multidisciplinary team, including orthopedic
and lateral. The medial compartment contains the oncologic surgeons, and musculoskeletal radiol-
abductor hallucis and flexor hallucis brevis mus- ogists and radiologists should consult with the
cles. The central compartment contains the flexor surgeon to determine the safe biopsy route (Liu
digitorum brevis, quadratus plantae, lumbrical, etal. 2007). An understanding of compartmental
and adductor hallucis muscles. The lateral com- anatomy is, therefore, essential to safely biopsy
partment contains the abductor and short flexor suspected soft tissue sarcomas (Toomayan etal.
muscles. 2005).
2.3Illustrations: Diagnostic Procedure 21
General principles for safe percutaneous biop- whenever possible because resection of these mus-
sies include the following: (1) the shortest path cles will result in a poor functional outcome. In the
between skin and the lesion should be chosen; (2) lower extremity, the vital structures include the
vital structures, such as neurovascular or joint knee joint capsule, greater trochanteric bursa, vas-
structures, should be avoided; (3) the needle path tus intermedius muscles, quadriceps tendon, tibial
must be in the same location where the incision for tubercle, peroneus brevis, peroneus longus distal
the definitive surgery will be made so that the nee- tendons, and several neurovascular bundles. If a
dle path can be excised. The needle should not tra- lesion is closely apposed to the femoral vessels, a
verse an uninvolved compartment, joint, or medial approach is preferred because a medial
neurovascular bundle; and (4) transverse surgical incision facilitates vessel exploration. The hand
biopsy incisions should be avoided because further and foot regions have such complex anatomies
resection is required upon definitive longitudinal that it is necessary to discuss with a surgeon in
incision (Bancroft etal. 2007; Liu etal. 2007; planning a biopsy route (Anderson etal. 1999;
UyBico etal. 2012). Several recommended needle UyBico etal. 2012). Knowledge of compartmental
paths for planning percutaneous needle biopsies of anatomy is important for optimal management of
potential sarcomas have been proposed for spe- sarcomas and must be considered when plan-
cific regions. These biopsy guidelines are based on ningand performing percutaneous needle biop-
the standard surgical approaches used for limb sal- sies. Radiologists performing needle biopsy for
vage surgery of the extremities. Lesions in the potential sarcomas should be aware of potential
upper arm should be biopsied through the anterior complications that can worsen the prognosis of
third of the deltoid muscle if possible. As the del- patients and alter treatment plans (Bancroft etal.
toid muscle is innervated by the axillary nerve 2007).
from posterior to anterior, if the needle passes
through the posterior portions of the deltoid mus-
cle, which is then resected, the residual anterior 2.3 Illustrations: Diagnostic
deltoid muscle will be denervated after resection Procedure
of the posterior portions of the muscle. In the pel-
vic region, the gluteal m
uscles should be preserved 2.3.1 Anatomic Compartments
a b
Anterior
Median nerve
Ulnar nerve
Posterior
Radial nerve
Fig. 2.1 The upper arm contains two compartments: anterior (solid line) and posterior (dashed line) in an axial
T1-weighted image (a) and a diagrm (b)
Median n.
a b
Radial n. Ulnar n.
Volar
Anterior interosseous n.
Mobile wad
Dorsal
Posterior interosseous n.
Fig. 2.2 The forearm contains three compartments: volar (solid line), dorsal (dashed line), and mobile wad (dotted
line) in an axial T1-weighted image (a) and a diagrm (b)
a b Femoral n.
Sartorius
Anterior
Gracilis
Medial
Gluteus max. Posterior
Sciatic n.
c d
Anterior
Sartorius
Medial Gracilis
Posterior
Sciatic n.
Fig. 2.3 The thigh contains three compartments: anterior (b, d, f). Images were obtained at upper thigh (a, b), mid-
(solid line), medial (dashed line), and posterior (dotted thigh (c, d) and distal thigh (e, f) levels
line) in axial T1-weighted images (a, c, e) and diagrams
e f
Anterior
Sartorius
Common Posterior
peroneal n. Medial
Tibial n. Gracilis
Fig. 2.3(continued)
a b
Anterior
Lateral
Tibial n.
Deep posterior
Posterior
Common
Peroneal n.
Sural n.
c d
Anterior
Deep posterior
Lateral
Tibial n.
Posterior
Sural n.
Fig. 2.4 The lower leg contains four compartments: T1-weighted images (a, c) and diagrams (b, d). Images
anterior (solid line), lateral (dashed line), deep posterior were obtained at upper lower leg (a, b) and mid-lower leg
(dotted line), and posterior (dash-dot line) in axial (c, d) levels
a b
Dorsal
Medial
Central
Lateral
Fig. 2.5 The sole of the foot is divided into three compartments: medial (dash-dot line), central (dashed), and lateral
(solid) in an axial T1-weighted image (a) and a diagram (b). The dorsum of the foot is considered extracompartmental
2.3.2 General Principles ofImage-Guided Percutaneous Biopsy
1
2
Fig. 2.6 Recommended biopsy needle path to avoid

the vital structures. To biopsy the mass in the left
adductor muscle, the shortest path between the skin
and the lesion (path 1) should be chosen. The needle
should not traverse an uninvolved compartment (star)
or femoral neurovascular bundle (arrowheads) (path 2)
a b
c d
Fig. 2.7 To biopsy the mass in the right vastus lateralis muscle, transverse biopsy path (a, b) should be avoided because
further resection is required upon definitive longitudinal incision (c, d)
Fig. 2.8 Lesions in the upper arm should be biopsied

through the anterior third of the deltoid muscle (path 1)
if it is possible because the deltoid muscle is innervated
by the axillary nerve from posterior to anterior
a b
Fig. 2.9 There is a necrotic lymph node in the subcutaneous layer of the thigh (a). Biopsy should be performed in the
solid portion (arrowhead) of the lymph node to obtain the sufficient tissue for histologic examination (b)
ing surgery. Radiographics. 2007;27(1):189205.

References doi:10.1148/rg.271065092.
Mankin HJ, Mankin CJ, Simon MA.The hazards
Anderson MW, Temple HT, Dussault RG, Kaplan of the biopsy, revisited. Members of the muscu-
PA.Compartmental anatomy: relevance to stag- loskeletal tumor society. JBone Joint Surg Am.
ing and biopsy of musculoskeletal tumors. AJR Am 1996;78(5):65663.
JRoentgenol. 1999;173(6):166371. doi:10.2214/ Toomayan GA, Robertson F, Major NM.Lower extremity
ajr.173.6.10584817. compartmental anatomy: clinical relevance to radiolo-
Bancroft LW, Peterson JJ, Kransdorf MJ, Berquist TH, gists. Skelet Radiol. 2005;34(6):30713. doi:10.1007/
OConnor MI.Compartmental anatomy relevant s00256-005-0910-2.
to biopsy planning. Semin Musculoskelet Radiol. UyBico SJ, Motamedi K, Omura MC, etal. Relevance
2007;11(1):1627. doi:10.1055/s-2007-984410. of compartmental anatomic guidelines for biopsy of
Liu PT, Valadez SD, Chivers FS, Roberts CC, Beauchamp musculoskeletal tumors: retrospective review of 363
CP.Anatomically based guidelines for core needle biopsies over a 6-year period. JVasc Interv Radiol.
biopsy of bone tumors: implications for limb-spar- 2012;23(4):5118. doi:10.1016/j.jvir.2012.01.058.
Staging ofSoft Tissue Sarcoma
3
Cancer staging is the process of determining how 3.1 Tumor (T-Staging)

much cancer is in the body and where it is located.
Staging describes the severity of an individuals can- The number of AJCC T stages for soft tissue
cer based on the magnitude of the original (primary) sarcoma of the trunk and extremities in eighth
tumor and the extent to which the cancer has spread edition (2016) has been increased from two (T1,
in the body. The stage of soft tissue sarcoma is the 5cm or less in greatest dimension and T2,
most significant factor in determining each patients greater than 5cm) to four, as follows: T1, 5cm
prognosis (the course of the disease and the chances or less in greatest dimension; T2, more than
of survival) and in selecting treatment options. 5cm and less than or equal to 10cm; T3, more
Although there are several staging systems for soft than 10cm and less than or equal to 15cm; and
tissue sarcomas, staging system of the American T4, more than 15cm. Some tumors, such as
Joint Committee on Cancer (AJCC) has generally pleomorphic sarcoma and myxofibrosarcoma,
been considered the standard (Table3.1) (Gibbs often have tail-like projections extending for a
etal. 2016; Fisher etal. 2016; Crago and Lee 2016; considerable distance along fascial and neuro-
Pollock and Maki 2016; Massarweh etal. 2015). vascular planes. Surrounding reactive edema, if
Compared to the standard tumor, node, and metasta- present, should not be included in the measure-
sis (TNM) factors prevalent in all AJCC solid organ- ment. In comparison to the seventh edition,
staging systems, a unique characteristic of the soft superficial versus deep location has been
tissue sarcoma system is the incorporation of histo- removed as part of the T criteria in the eighth
logic grade. It is applicable to all soft tissue sarcoma edition. For completeness, depth is evaluated
subtypes, except desmoid tumor/deep fibromatosis relative to the investing fascia, and superficial is
and Kaposi sarcoma. In the eighth edition, staging defined as the lack of any involvement of super-
criteria has been specifically separate on the ana- ficial investing muscular fascia. For staging,
tomic primary site of the soft tissue sarcoma as fol- non-superficial head and neck, intrathoracic,
lows: (1) extremity and trunk, (2) retroperitoneum, intra-abdominal, retroperitoneal, and visceral
(3) head and neck, and (4) visceral sites. lesions were considered deep lesions.

28 3 Staging ofSoft Tissue Sarcoma
Table 3.1 TNMG staging for soft tissue sarcomas of the trunk and extremities according to the AJCC staging
manuala
Primary tumor (T)
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
T1 Tumor 5cm in greatest dimensiona
T2 5cm<tumor 10cm
T3 10cm<tumor 15cm
T4 Tumor >15cm in greatest dimensiona
Regional lymph nodes (N)
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
Distant metastasis (M)
M0 No distant metastasis
M1 Distant metastasis
Histologic grade (G)
GX Grade cannot be assessed
G1 Grade 1
G2 Grade 2
G3 Grade 3
AJCC prognostic stage groups

Stage IA T1 N0 M0 G1, GX
Stage IB T2 N0 M0 G1, GX
T3 N0 M0 G1, GX
T4 N0 M0 G1, GX
Stage II T1 N0 M0 G2, G3
Stage IIIA T2 N0 M0 G2, G3
Stage IIIB T3 N0 M0 G2, G3
T4 N0 M0 G2, G3
Stage IV Any T N1 M0 Any G
Any T Any N M1 Any G
Adapted from AJCC: Soft tissue sarcoma. IN: Yoon SS, Maki RG, Asare EA, etal., eds. AJCC Cancer Manual. 8th ed.
a
NewYork: Springer; 2016:507515
3.2 Nodes (N-Stage) sidered to occur through hematogenous and,

less commonly, lymphatic spread. Several sub-
Regional and/or distant metastatic dissemina- types with the highest rates of lymph node
tion of soft tissue sarcomas is generally con- metastasis are as follows: alveolar/embryonal
3.6 Illustrations: Staging of Soft Tissue Sarcoma 29
rhabdomyosarcoma, clear cell sarcoma, syno- to assign a grade of 1 (low grade) through 3
vial sarcoma, epithelioid sarcoma, and angio- (high grade). Accurate grading requires an
sarcoma (Fong etal. 1993; Mazeron and Suit adequate sample of well-fixed tissue for evalu-
1987). ation and may not always be possible based on
needle biopsies or in tumors that were previ-
ously treated with radiation or chemotherapy.
3.3 Metastasis (M-Stage) Core biopsy with image guidance is thought to
be the most accepted method for the initial
Metastasis from soft tissue sarcomas may sampling of suspected sarcomas (Gibbs etal.
occur to distant organs or present as lesions 2016).
that are distant from the primary tumor con-
tained within the same anatomic compartment.
Metastatic sites for soft tissue sarcoma often 3.5 Proposed Report Format
depend on the original site of the primary
lesion. Pulmonary metastases are the most (1) Primary tumor
common manifestations of distant disease for (a) MR imaging signal or CT attenuation
patients with extremity sarcoma, whereas the characteristics
liver is the most common location for patients (b) Extent and location of necrosis within
with retroperitoneal and gastrointestinal sar- the tumor
coma (Crago and Lee 2016). (c) Location in extremity, including rela-
tionship to superficial fascia
(d) Presence and location of tumor tails
3.4 Histologic Grade (G-Stage) (e) Size (in three dimensions)
(2) Local extent
The histologic grade is the most important (a) Invasion of muscles, bones, and joints
component of staging and is itself a predictor (b) Contact with, or encasement of, blood
of metastatic disease. In line with the FNCLCC vessels and nerves
(Fdration Nationale des Centres de Lutte (c) Extension into the lumen of blood
Contre la Cancer, French Federation of Cancer vessels
Centers) sarcoma grading system, points are (d) Presence of nearby satellite nodules
assigned using a scoring system ranging from (3) Regional lymph node involvement
zero to three. Three distinct histologic grades
are recognized based on the degree of tumor
differentiation, mitotic activity or rate, and 3.6 I llustrations: Staging ofSoft
degree of necrosis (Massarweh etal. 2015; Tissue Sarcoma
Coindre 2006; Guillou etal. 1997; Trojani
etal. 1984) (Table3.2). The total score is used 3.6.1 Stage I
30
Table 3.2 FNCLCC grading system: Parameters for grading

Score
Histologic features 0 1 2 3
Tumor differentiation Sarcomas closely Sarcomas for which histologic typing is Embryonal and undifferentiated sarcomas, sarcomas of
resembling normal adult certain, doubtful type, synovial sarcomas, extraskeletal
mesenchymal tissue, e.g., myxoid liposarcoma, fibrosarcoma, osteosarcomas, Ewing sarcoma/PNET of soft tissue,
e.g., well-differentiated myxofibrosarcoma, pleomorphic MFH e.g., round cell/pleomorphic/dedifferentiated
(liposarcoma, with storiform pattern, conventional liposarcoma, undifferentiated pleomorphic sarcoma,
fibrosarcoma, MPNST, leiomyosarcoma, well-differentiated/ poorly differentiated/epithelioid MPNST, conventional
leiomyosarcoma) conventional angiosarcoma, well- malignant solitary fibrous tumor, poorly differentiated/
differentiated solitary fibrous tumor pleomorphic/epithelioid leiomyosarcoma, pleomorphic
rhabdomyosarcoma, extraskeletal myxoid
chondrosarcoma, mesenchymal chondrosarcoma,
poorly differentiated/epithelioid angiosarcoma, ASPS,
epithelioid sarcoma, clear cell sarcoma, malignant
rhabdoid tumor, undifferentiated sarcoma, NOS
Mitotic activity 09 mitoses per 10 HPFa 1019 mitoses per 10 HPF 20 mitoses per 10 HPF
Tumor necrosis No necrosis <50% tumor necrosis 50% tumor necrosis
Histologic grade (G) Total score

Grade 1 23
Grade 2 45
Grade 3 68
Adapted from Guillou L, Coindre JM, Bonichon F, etal. (Guillou etal. 1997) Comparative study of the National Cancer Institute and French Federation of Cancer Centers
Sarcoma Group grading systems in a population of 410 adult patients with soft tissue sarcoma. J Clin Oncol 1997; 15(1):35062; and Coindre JM.Grading of soft tissue sarco-
mas: review and update. Arch Pathol Lab Med 2006; 130(10):1449
a
HPF measures 0.1734mm2
3 Staging ofSoft Tissue Sarcoma
a b
Fig. 3.1 A 35-year-old female with well-differentiated (not shown) (N0, M0). FNCLCC grade is 1: tumor dif-
liposarcoma. On coronal T2WI (a) and axial T1WI (b) of ferentiation, well-differentiated (score 1); mitosis count,
the left thigh, the mass measured 17cm at its greatest <1/10 HPF (score 1); and tumor necrosis, 0% (score 0).
dimension (T4). There is no evidence of regional node This patient was ultimately diagnosed with stage IB
metastasis or distant metastasis on other imaging work-up liposarcoma
3.6.2 Stage II
a c
Fig. 3.2 A 62-year-old female with leiomyosarcoma. On gland is confirmed as benign thyroid pathology. FNCLCC
sagittal (a) FS and axial (b) T2WI of the left lower leg, the grade is 2: tumor differentiation, spindle cell/pleomorphic
tumor is smaller than 5.0cm at its greatest dimension (score 3); mitosis count, 6/10 HPF (score 1); and tumor
(T1). There is no evidence of regional node metastasis or necrosis, 0% (score 0). This patient was ultimately diag-
distant metastasis on PET (c) (N0, M0). A very small nosed with stage II leiomyosarcoma
hypermetabolic lesion in the right lobe of the thyroid
3.6.3 Stage III
a b
Fig. 3.3 A 57-year-old male with myxofibrosarcoma. mass measures 9.3cm at its greatest dimension (T2).
Coronal T1WI (a) of the thigh reveals a circumscribed, There is no evidence of regional node metastasis or dis-
isointense (relative to skeletal muscle) soft tissue mass tant metastasis (N0, M0). FNCLCC grade is 2: tumor dif-
beneath the superficial fascia. The lesion is bright hyper- ferentiation, score 2; mitosis count, 3/10 HPF (score 1);
intense on coronal FS T2WI (b) and has inhomogeneous and tumor necrosis, 0% (score 0). This patient was ulti-
intense contrast enhancement on C+ FS T1WI (c). The mately diagnosed with stage IIIA myxofibrosarcoma
a b
Fig. 3.4 A 32-year-old male with myxoid/round cell (c). The mass measures 12cm at its greatest dimension
liposarcoma. Coronal T1WI (a) of the thigh shows an (T3). There is no evidence of regional node metastasis or
isointense intramuscular soft tissue mass with several thin distant metastasis on other imaging work-up (not shown)
hyperintense rims or lines (arrows). Coronal FS T2WI (b) (N0, M0). FNCLCC grade is 3: tumor differentiation,
shows the mass to be fluid-equivalent hyperintense, with a round cell (score 3); mitosis count, 13/10 HPF (score 2);
signal drop of T1 hyperintense areas. The lesion exhibits and tumor necrosis, 5% (score 1). Ultimately, this patient
heterogeneous contrast enhancement on coronal C+ T1WI was diagnosed with stage IIIB liposarcoma
3.6.4 Stage IV
a c
Fig. 3.5 A 62-year-old female with leiomyosarcoma. On metastasis in the right middle lobe on chest CT (b) and
sagittal T2WI of the right thigh (a), the mass measures PET (c). Irrespective of any T-stage, N-stage, and histo-
11.0cm at its greatest dimension and is deeply located logic grade, this patient was diagnosed with stage IV
beneath the superficial fascia (T3). Note the pulmonary leiomyosarcoma
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P, Collin F, Vilain MO, Mandard AM, Le Doussal V,
Part II
WHO Classification of Soft Tissue Tumors
and Radiologic Illustrations
Adipocytic Tumors
4
Benign lipomatous lesions represent a variety of metastasizes in only 1520% of cases, mortality
adipose tumors and include lipoma, lipomatosis, being most often related to uncontrolled local
lipomatosis of nerve, lipoblastoma/lipoblastoma- recurrences. The clinical behavior of myxoid
tosis, angiolipoma, myolipoma of soft tissue, liposarcoma is determined primarily by histolog-
chondroid lipoma, spindle cell lipoma/pleomor- ical grade (manifested by the degree of hyper-
phic lipoma, and hibernoma. Benign lipomatous cellularity). High-grade (formerly round cell)
lesions often have characteristic imaging fea- myxoid liposarcoma exhibits a remarkable ten-
tures, including diffuse or focal areas that are dency to metastasize to bone and soft tissues.
similar or identical to subcutaneous fat. These Pleomorphic liposarcoma shares highly aggres-
imaging characteristics and lesion extent are best sive clinical behavior with other pleomorphic
appreciated with MR imaging. sarcomas (Dei Tos 2014).
Malignant adipocytic tumors account for The MR imaging features of liposarcomas
1035% of all soft tissue sarcomas. In fact, lipo- vary according to the wide spectrum of patho-
sarcomas represent a heterogeneous group of dis- logic appearances. Imaging findings contribute to
tinctive lesions that pose several diagnostic the final histologic diagnosis of these tumors and
difficulties. The World Health Organization can be used to orientate the biopsy, which should
(WHO) Committee for the Classification of Soft be performed on both the lipomatous and nonli-
Tissue Tumors in 2013 categorized liposarcomas pomatous components (El Ouni etal. 2010).
into one intermediate and three malignant sub-
types, including atypical lipomatous tumor/well-
differentiated liposarcoma, dedifferentiated 4.1 Lipoma
liposarcoma, myxoid liposarcoma, and pleomor-
phic liposarcoma (Murphey etal. 2005; Dei Tos Lipoma is the most common benign soft tissue
2014). tumor, is composed of mature fat cells, and is not
The exact classification of the liposarcoma distinguishable histologically from normal fat. It
subtypes is crucial for decision-making in is either a superficial or deep-seated mass, com-
patients given that the aggressiveness of local and monly in subcutaneous fat, muscles, or intermus-
systemic treatment modalities may vary substan- cular planes. Most lipomas are discrete masses;
tially (Henze and Bauer 2013). Atypical lipoma- however, superficial lipomas can blend imper-
tous tumor/well-differentiated liposarcoma is a ceptibly with the surrounding subcutaneous fat.
locally aggressive neoplasm that is virtually inca- Intramuscular lipomas can have ill-defined mar-
pable of systemic spread. Dedifferentiated lipo- gins due to fat infiltration among muscle fibers in
sarcoma, despite its high-grade morphology, deep-seated lesions (Bancroft etal. 2013).

40 4 Adipocytic Tumors
At US, lipomas are hyperechoic masses, occa- 4.3 Lipomatosis ofNerve

sionally with heterogeneity caused by septa or
nonadipose tissues. No posterior sonic enhance- Lipomatosis of nerve (LON) is a benign tumor
ment is observed. The MR imaging findings of involving peripheral nerve that has been described
ordinary lipoma are well described, with high by various other names, including fibrolipoma-
signal intensity on both T1- and T2-weighted tous hamartoma, neural fibrolipoma, and fibro-
images and a signal drop on fat-suppressed fatty proliferation of a nerve. The histologic
images. The same pattern is observed for subcu- evaluation demonstrates diffuse nerve enlarge-
taneous fat. Signal alteration is caused by infarc- ment secondary to interfascicular fibrofatty infil-
tion, hemorrhage, inflammation, and ossification tration. LON most commonly occurs in the
or in cases when lipomas admix with other mes- median nerve and has been reported in multiple
enchymal elements. In these cases, lipomas con- other nerve distributions. LON is frequently
tain areas with a decreased signal intensity on associated with varying degrees of localized mes-
T1-weighted images and various signal intensi- enchymal overgrowth in the territory of nerve
ties on T2-weighted images (Drevelegas etal. distribution. This overgrowth ranges from mini-
2004). When a lipoma occurs adjacent to cortical mal digital enlargement to extensive bone and
bone, it is commonly associated with reactive soft tissue hypertrophy, which is referred to as
changes in the underlying cortex. Lipomas fre- macrodystrophia lipomatosa (Gupta etal. 2016).
quently demonstrate intrinsic thin septa (< 2mm) The MR imaging appearance of LON is
on CT or MR images. The presence of only thin unique and characteristic, obviating the need for
delicate septa, particularly when few in number, biopsy for diagnosis. Serpentine, low-intensity
favors the diagnosis of soft tissue lipoma over structures, representing thickened nerve fasci-
well-differentiated liposarcoma (Murphey etal. cles, are surrounded by or embedded in evenly
2004). distributed fat, which appears on MR imaging as
high signal intensity on T1-weighted images and
low signal intensity on STIR and fat-suppressed
4.2 Lipomatosis T2-weighted images. A coaxial-cable-like
appearance in the short-axis plane and a spaghetti-
Lipomatosis represents a diffuse overgrowth of like appearance in the long-axis planes are the
mature adipose tissue. The multiple types of lipo- most typical findings for this tumor (Van
matosis are typically differentiated by anatomic Breuseghem etal. 2003).
location, and varying clinical manifestations
have been described. These manifestations
include multiple symmetric lipomatosis 4.4 Lipoblastoma/
(Madelungs disease), infiltrating congenital Lipoblastomatosis
lipomatosis of the face, encephalocraniocutane-
ous lipomatosis, shoulder girdle lipomatosis, adi- Lipoblastoma is a lipoma variant of the embry-
posis dolorosa, pelvic lipomatosis, and onic lipoblast and is categorized into two forms
mediastinal lipomatosis. The extensive involve- as follows: (1) a localized, superficial, and well-
ment of both muscle and subcutaneous tissue circumscribed or encapsulated form termed lipo-
typically differentiates a large intermuscular or blastoma and (2) a diffuse, multicentric,
intramuscular lipoma from lipomatosis. Imaging, unencapsulated, and infiltrative form termed
particularly CT and MR imaging, reveals these lipoblastomatosis. Lipoblastomas occur most fre-
infiltrative fatty masses, typically in a subcutane- quently in male children younger than 3years of
ous distribution but more frequently between the age and have been reported to arise where imma-
deep muscles (Murphey etal. 2004). ture adipose elements persist, namely, the neck,
4.7Hibernoma 41
axilla, and prevertebral soft tissues (Salem etal. 4.6 Spindle Cell Lipoma
2011).
The imaging of lipoblastomas reflects their Spindle cell lipoma is a benign lesion in which
histopathology, which typically shows an mature fat is replaced by a mixture of mature adi-
admixture of mature and immature adipocytes, pocytes and collagen-forming spindle cells.
often with a prominent myxoid matrix. MR These lesions are usually located in the subcuta-
imaging usually precisely depicts the well-cir- neous tissues of the shoulder, neck, and upper
cumscribed margins of the lipoblastoma, back and can exhibit intramuscular extension. A
although the margins of lipoblastomatosis may lipoma with a spindle cell subtype can represent
be difficult to identify. CT and MR imaging a diagnostic challenge to radiologists, patholo-
show a predominantly fatty mass, with the non- gists, and surgeons given its frequent presenta-
lipomatous areas of the mass showing a nonspe- tion as a lesion containing little or no macroscopic
cific imaging appearance. The imaging or microscopic fat (Khashper etal. 2014).
appearance of lipoblastomas may simulate that The imaging features of spindle cell lipoma
of a liposarcoma; however, such lesions are depend on their adipose and nonadipose contents.
exceedingly rare in children (Bancroft etal. The MR examination grading of adipose: nonadi-
2006). The age of the patient is therefore the pose content of spindle cell lipomas correlates with
most useful distinction as there are no known the histological grading of the excision specimen.
imaging findings which separate these two enti- The non-adipocytic areas are composed of spindle
ties (Moholkar etal. 2006). cells in a fibromyxoid background, which corre-
spond to the low T1 signal and enhance following
administration of intravenous contrast material
4.5 Angiolipoma (Kirwadi etal. 2014). Because of the similarity of
the imaging features with liposarcoma, preoperative
Angiolipomas typically present as small (< 2cm image-guided biopsy is often required.
in diameter), painful subcutaneous masses. They
most commonly affect young male patients in the
second to third decades of life. The most com- 4.7 Hibernoma
mon locations in order of frequency are the fore-
arm, trunk and upper arm. Multiple angiolipomas Hibernoma is a rare benign adipose tumor that is
are reported in approximately 70% of cases. composed of brown fat-like multivacuolated
The MR imaging features of angiolipoma are cells, univacular cells resembling lipocytes and
the presence of fat nodules with or without areas lipid-free cells with eosinophilic granular cyto-
of low signal intensity on T1- and T2-weighted plasm. Hibernomas have a variable histological
images. The location of the low-signal-intensity appearance depending on the relative amounts of
areas vary, being either in the peripheral or cen- multivacuolated brown fat cells associated with
tral portion of the mass. On histopathologic capillary proliferation and fibrovascular septa.
examination, the low-signal-intensity areas on Compared to the subcutaneous fat, hibernomas
T1- and T2-weighted images correspond to are slightly hyperdense, with attenuation values
areas of dense capillary proliferation. Only very between those of fat and skeletal muscle on CT.On
small portions of the low-signal-intensity areas MR imaging, these tumors are slightly hypoin-
on T1-weighted images appear as high-signal- tense relative to fat on T1-weighted imaging and
intensity areas on T2-weighted images. show incomplete signal suppression on fat-sup-
(Kitagawa etal. 2014). Those vascular compo- pressed T2-weighted imaging. Hibernomas often
nents markedly enhance with intravenous con- demonstrate prominent internal vascularity with
trast material. variable tumoral enhancement. Lipoma-like
h ibernomas are usually isointense with subcutane- occurs in the extremities and then in the retro-
ous fat on T1-weighted images. In contrast, non- peritoneum. Histologically, this tumor is charac-
lipoma-like hibernomas are predominantly slightly terized by the presence of mature adipocytes of
hypointense to subcutaneous fat on T1-weighted irregular size arranged in bands that are separated
images, often displaying marked or moderate by thick fibromyxoid septa. These septa charac-
inhomogeneity, with prominent septa and vessels teristically comprise cells with atypical hyper-
(Ritchie etal. 2006). Some findings may overlap chromatic nuclei that exhibit overexpression of
with other, more common, benign or low-grade MDM2 and CDK4 (Kind etal. 2009).
lipomatous neoplasms, such as lipoma and atypi- At imaging, ATLs/WDLPSs resemble lipo-
cal lipomatous tumor (ALT), leading to diagnostic mas and have a characteristic appearance as a
difficulty (Liu etal. 2013). Because of their ability mass that is predominantly composed of highly
to produce heat and exhibit thermoregulation, differentiated adipose tissue. Nodular or globular
intense uptake of FDG can be observed on PET. nonlipomatous components can be observed,
which are usually less than 2cm in size. ATLs/
WDLPS is distinguished from benign lipoma by
4.8 Lipoma Arborescens variation in adipocytic size and the presence of
cytologic atypia. Consequently, radiological dif-
Lipoma arborescens is a rare intra-articular ferentiation of ATLs/WDLPS from lipoma can
lipoma-like lesion characterized by fatty infiltra- be challenging. MR imaging of the internal char-
tion of the subsynovial connective tissue. This acteristics is useful for the differential diagnosis
frond-like lesion receives its name from its of these tumors. When a predominantly adipose
resemblance to a tree in leaf. Lipoma arborescens mass demonstrates multiple thick (> 2mm) septa
is generally considered one of the rarest of syno- with marked enhancement, it is more likely
vial proliferative lesions (Garner and Bestic ATLs/WDLPS than lipoma (Ohguri etal. 2003).
2013). The knee is the most common site of Other factors favoring the diagnosis of ATLs/
involvement, although the glenohumeral joint, WDLPS over lipoma include male sex, age
subdeltoid bursa, hip, and elbow can be affected. greater than 66years, lower percentage of fat
Lipoma arborescens has pathognomonic MR (less than 75% of the lesion), the presence of cal-
imaging findings that include a frond-like syno- cification, and lesion size greater than 10cm
vial mass that is isointense relative to fat with all (Kransdorf etal. 2002).
sequences (including fat-suppression sequences), The sclerosing variant of well-differentiated
joint effusion, and a lack of magnetic susceptibil- liposarcoma should be included in the differen-
ity artifact. Three distinct patterns of lipoma tial diagnosis of any well-circumscribed lipoma-
arborescens have been identified, the most com- tous mass containing variable amounts of
mon of which is a diffuse villous proliferation, nonlipomatous elements, particularly when
although a discrete mass lesion and a mixed pat- located in the retroperitoneum. The sclerosing
tern can also be observed (Coll etal. 2011). Bone variant has a tumor composition ranging from
erosions in association with lipoma arborescens predominantly fatty to entirely devoid of macro-
may develop in relatively tight joints, such as scopic fat and may be associated with an
the glenohumeral joint (Chae etal. 2009). increased propensity for dedifferentiation (Bestic
etal. 2013).
4.9 typical Lipomatous Tumor/

A
Well-Differentiated 4.10 Dedifferentiated
Liposarcoma Liposarcoma
Atypical lipomatous tumor or well-differentiated Dedifferentiated liposarcoma (DDLPS) is a sub-

liposarcoma (ATL/WDLPS) is the most common type of liposarcoma, being a transition from low-
liposarcoma. ATLs/WDLPS most frequently grade to high-grade non-lipogenic sarcoma. This
4.11 Myxoid Liposarcoma 43
tumor is characterized histologically by two myxoid liposarcomas often pathognomonically

components as follows: one well-differentiated present with low signal intensity on T1-weighted
liposarcoma-type component and another com- MR images and marked high signal intensity on
ponent composed of undifferentiated sarcoma- T2-weighted MR images. Most myxoid liposar-
type cells. The cells also exhibit overexpression comas have lacy or linear, amorphous foci of fat.
(immunohistochemistry) and amplification The fat also typically constitutes only a small vol-
(FISH) of MDM2 and CDK4 (Kind etal. 2009). ume of the overall mass size (<10% of the lesion)
The imaging findings of DDLPS are similar and is often observed in septa (lacy or linear pat-
with WDLPS and are best appreciated on MR tern) or as subtle, small nodules in the lesion
imaging. A nonlipomatous mass (intermediate- (Murphey etal. 2005). The use of fat suppression
to-
hypointense T1 signal, intermediate-to- can be helpful to further increase confidence in
hyperintense T2 signal) arising within or the detection of adipose content and differentiate
adjacent to a WDLPS is suspicious for dedif- fat from hemorrhagic components. Some myxoid
ferentiation (Gupta etal. 2016). The imaging liposarcomas appear to be cystic at unenhanced
differential diagnosis for a focal nodular nonli- MR imaging, although they enhance similarly to
pomatous component within a well-differenti- other solid masses on contrast- enhanced MR
ated liposarcoma includes a region of imaging (Sung etal. 2000). The following three
collagenized tissue, metaplastic mineraliza- patterns of enhancement are observed: solid, nod-
tion, a region of dedifferentiation, and fat ular enhancement; peripheral and septal enhance-
necrosis. It is important to recognize these ment; and uniform, central enhancement with
foci, and the biopsy must be directed at both non-enhancing septations (Wortman etal. 2016a).
the lipomatous and nonlipomatous components Myxoid liposarcomas with more prominent round
to ensure accurate pathologic diagnosis cell components may demonstrate areas of rela-
(Murphey etal. 2005). tively lower water content at MR imaging. These
areas may have nonspecific features of solid tis-
sue with intermediate signal intensity on T1- and
4.11 Myxoid Liposarcoma T2-weighted MR images. Sonographic evaluation
can be helpful in cyst-like myxoid liposarcomas
Myxoid liposarcoma is the second largest group on MR imaging by demonstrating that the mass is
of malignant adipocytic neoplasms, accounting not truly a cyst.
for approximately 3035% of all liposarcomas. Extremity myxoid liposarcomas are most fre-
Round cell liposarcoma is defined as a form of quently intermuscular lesions (7080% of cases),
myxoid liposarcoma that has an associated and an origin in muscle or subcutaneous location
round cell component in greater than 5% of the is less common. Myxoma is another diagnostic
tumor. Previously, myxoid and round cell lipo- consideration, although myxomas are generally
sarcomas were considered distinct histologic intramuscular lesions with surrounding muscle
subtypes. However, the WHO classification of atrophy and edema, all of which are manifesta-
soft tissue tumors has now combined these tions not observed in myxoid liposarcoma
lesions as myxoid liposarcoma, representing a (Murphey etal. 2005).
continuum of these two previous subtypes Presentation with either primary or recurrent
(Murphey etal. 2005). At histologic analysis, disease, tumor size, and tumor grade are of major
myxoid liposarcomas consist of a myxoid importance. Complete tumor resection with neg-
matrix as the predominant component and small ative margins should be the objective of surgery
amounts of mature fat. with an evident impact on local control. Myxoid
A spectrum of MR imaging abnormalities is liposarcoma is known to be particularly sensitive
observed in myxoid liposarcomas. These findings to radiotherapy, and MR imaging often shows a
depend on the amount of fat and myxoid tissue, decrease in size and enhancement and increase in
the cellularity and vascularity, and the presence of internal fat following radiotherapy (Wortman
necrosis. Because of their high water content, etal. 2016b).
4.12 Pleomorphic Liposarcoma (29%). The imaging appearance of pleomorphic

liposarcoma overlapped most frequently with high-
Pleomorphic liposarcoma is a high-grade liposar- grade myxoid liposarcoma: distinguishing factors
coma and the least common subtype of liposar- included the presence of thick septations and homo-
coma. It occurs in the extremities and, less geneity on T2-weighted images in high- grade
commonly, in the trunk and retroperitoneum. myxoid liposarcoma, which were very uncom-

Pleomorphic liposarcoma is difficult to differen- moncharacteristics in pleomorphic liposarcoma
tiate on MR imaging from other soft tissue sarcomas (Wortman etal. 2016a).
because it has nonspecific signal characteristics (T1
hypointensity and T2 hyperintensity) and because of
the paucity of fat. In one study using MR imaging, 4.13 Illustrations: Adipocytic
the tumor demonstrated peritumoral edema (93%), Tumors
necrosis (86%), intense enhancement (77%), intra-
tumoral hemorrhage (36%), and intratumoral fat 4.13.1 Lipoma
a b
Fig. 4.1 Intramuscular lipoma. Coronal T1WI (a) shows predominantly fatty mass and axial postcontrast FS T1WI
a subfascial fatty mass in the left vastus lateralis muscle, (c) shows a complete loss of signal. There are multiple
with diffuse T1 hyperintensity. Axial T1WI (b) shows a thin septa with T1 hypointensity and mild enhancement
4.13Illustrations: Adipocytic Tumors 45
a b
Fig. 4.2 Intermuscular lipoma. Coronal T1WI (a) shows mately 100 HU.Longitudinal extended-field-of-view
a fatty mass between the splenius capitis, levator scapu- US (c) shows a large intermuscular mass that is hyper-
lae, and trapezius muscles. Axial CT scan (b) shows a echoic relative to the adjacent musculature
well-demarcated mass with fat attenuation of approxi-
a b
Fig. 4.3 Parosteal lipoma. Right shoulder radiography scapula. There is a bony excrescence (arrowheads) aris-
(a) and coronal T1WI (b) show a low-density or T1 hyper- ing from the lateral border of the scapula
intense fatty mass (arrow) on the lateral aspect of the right
4.13.2 Lipomatosis
a b
Fig. 4.4 Lipomatosis. Coronal and axial T1WIs (a, b) show diffuse lipomatous infiltration in the back and right poste-
rior thigh subcutaneous layer
a b
Fig. 4.5 Lipomatosis. Coronal and axial T1WIs (a, b) show diffuse lipomatous infiltration in the left shoulder and
chest wall, affecting both muscles and subcutaneous tissues
4.13.3 Lipomatosis ofNerve
a b
Fig. 4.6 Lipomatosis of nerve. Coronal and axial T1WIs (a, b) show marked enlargement of the median nerve (arrow)
with diffuse fat infiltration, resulting in a spaghetti-like (a) or coaxial cable-like (b) appearance
a b
Fig. 4.7 Lipomatosis of nerve. Sagittal and axial T2WIs (a, b) show diffuse thickening of the tibial (arrow) and pos-
terior femoral cutaneous (arrowhead) nerves, with interfascicular fat deposition
4.13.4 Lipoblastoma
a b
c d
Fig. 4.8 Lipoblastoma. Axial T1WI (a) shows a fat- postcontrast FS T1WI (c) shows a largely non-enhancing
containing mass in the subcutaneous layer of the right mass. (d) Gross appearance of lipoblastoma with exten-
paramedian buttock. Axial FS T2WI (b) shows a large sive myxoid change
area of hyperintensity and saturated fat components. Axial
a b
Fig. 4.9 Lipoblastomatosis. Axial T1WI (a), T2WI (b) and coronal T1WI (c) show an infiltrative fatty lesion (arrow)
with intralesional streaks in the left pubic subcutaneous layer
Fig. 4.9(continued)
4.13.5 Angiolipoma
a b
Fig. 4.10 Angiolipoma. Axial T1WI (a) shows a perifas- hyperintense areas (arrowhead). Axial postcontrast FS
cial fatty mass (arrow) with heterogeneous signal inten- T1WI (c) shows heterogeneous enhancement with a small
sity on the anterior proximal lower leg. Axial FS T2WI intramuscular extension (arrow) into the tibialis anterior
(b) demonstrates suppressed fat components with some
Fig. 4.10(continued)
4.13.6 Spindle Cell Lipoma
a b
Fig. 4.11 Spindle cell lipoma. Axial T1WI (a) shows a nents (arrow). Axial postcontrast FS T1WI (c) shows het-
well-circumscribed subcutaneous mass in the posterior erogeneous enhancement in the nonadipose elements.
neck. The mass appears as a mixture of fat (arrow) and (d)Gross appearance of spindle cell lipoma showing a
nonadipose components. Sagittal FS T2WI (b) shows a predominantly fatty lesion without hemorrhage or
large area of hyperintensity and hypointense fat compo- necrosis
c d
4.13.7 Hibernoma
a b
Fig. 4.12 Hibernoma. Axial T1WI (a) shows a subcuta- Axial FS T2WI (b) and postcontrast FS T1WI (c) show a
neous mass slightly hypointense to the surrounding fat large area of T2 hyperintensity or prominent contrast
tissues in the left posterior upper arm. There are linear or enhancement
dot-like structures (arrows) in keeping with blood vessels.
a b
c d
Fig. 4.13 Hibernoma. Precontrast CT scan (a) shows a (arrow), with hypoattenuating components. (c) Left ninth
left posterior mediastinal mass (arrow) with heteroge- intercostal arteriogram demonstrates marked vascularity
neous low attenuation (mean HU 10). Postcontrast CT of the tumor. (d) Cut section of the mass, showing a yel-
scan (b) shows prominent enhancement of the mass lowish solid mass with streaky areas of myxoid changes
4.13.8 Lipoma Arborescens
a b
Fig. 4.14 Lipoma arborescens. Oblique coronal T1WI coronal postcontrast FS T1WI (c) shows prominent syno-
and FS T2WI (a, b) show lipomatous villous proliferation vial enhancement (arrows) lining over the villous subsy-
(arrows) within the subacromial subdeltoid bursa. Oblique novial fat proliferation
4.13.9 Well-Differentiated Liposarcoma
a b
Fig. 4.15 Well-differentiated liposarcoma. Axial T1WI terior thigh. There are multiple septa (arrows) with
and postcontrast FS T1WI (a, b) show a very large lipo- prominent enhancement
matous mass in the intermuscular planes of the right pos-
a b
Fig. 4.16 Well-differentiated liposarcoma. Coronal T1WI and STIR (a, b) show a huge fatty mass (arrows) in the left
posterior thigh. An intratumoral, non-enhancing geographic area of fat necrosis (arrowheads) is seen
a c
Fig. 4.17Well-differentiated liposarcoma, sclerosing ponents (arrow). Coronal postcontrast FS T1WI (c) shows
variant. Axial T1WI and T2WI (a, b) show an intermus- prominent enhancement of the nonlipomatous elements
cular fatty mass with hazy and streaky nonadipose com- (arrows)
4.13.10 Dedifferentiated Liposarcoma
a b
Fig. 4.18 Dedifferentiated liposarcoma. Axial T1WI and postcontrast FS T1WI (a, b) show an intramuscular fatty
tumor with an enhancing nonlipomatous mass (arrow) in the left anterior thigh
a b
Fig. 4.19 Dedifferentiated liposarcoma. Axial T1WI and differentiated liposarcoma. (c) Gross appearance of
postcontrast FS T1WI (a, b) show diffuse fatty replace- dedifferentiated liposarcoma showing a distinct margin
ment of the posterior compartment (arrowheads) of the (arrowheads) between well-differentiated and dedifferen-
right thigh. A vividly enhancing mass of high-grade sar- tiated zones
coma is seen with abrupt transition (arrows) from well-
a b
Fig. 4.20 Dedifferentiated liposarcoma. Axial T1WI (a) and nonadipose components. Coronal FS T2WI (b) shows
shows a soft tissue mass (arrows) between the serratus a hyperintense nature of the mass with central heteroge-
anterior and the rib cage. The mass consists of fat tissue neous signal intensity
4.13.11 Myxoid Liposarcoma
a b
Fig. 4.21 Myxoid liposarcoma. Axial T1WI (a) shows a hyperintense myxoid tumor with an area of fat suppres-
subfascial mass in the vastus lateralis muscle with T1 sion (arrow). Axial postcontrast FS T1WI (c) shows
hypointensity. Small area of fat (arrow) is seen on the prominent enhancement
anterior side of the mass. Axial FS T2WI (b) shows a
a b
Fig. 4.22 Myxoid liposarcoma. Coronal T1WI (a) shows keeping with myxoid tumor. Fatty areas (arrows) are of
a large lobulated mass in the left distal posterior thigh relatively low signal intensity due to fat suppression.
intermuscular fat plane. There are T1 hypointense (myx- Coronal postcontrast FS T1WI (c) shows heterogeneous
oid tissue) and hyperintense (fat components, arrows) and intense enhancement
areas. Coronal FS T2WI (b) shows bright high signal in
a b
Fig. 4.23 Myxoid liposarcoma. Axial T1WI (a) shows a (b) shows a cyst-like T2 hyperintense mass. Axial post-
subfascial mass isointense to muscle in the gracilis mus- contrast FS T1WI (c) shows homogeneous enhancement
cle. There is no discernible intratumoral fat. Axial T2WI suggestive of myxoid tumor rather than cystic mass
4.13.12 Pleomorphic Liposarcoma
a b
Fig. 4.24 Pleomorphic liposarcoma. Axial T1WI and rior part of nonspecific signal. Axial postcontrast FS
T2WI (a, b) show an intramuscular mass of the peroneus T1WI (c) shows more prominent enhancement on the pos-
longus in the left proximal lower leg. The mass consists of terior portion of the tumor than on the anterior fat-
the anterior half of fat-like signal (arrow) and the poste- containing area (arrow)
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Fibroblastic/Myofibroblastic
Tumors 5
Fibroblastic and myofibroblastic tumors are a nosed as a sarcoma. It usually presents as a

group of mesenchymal tumors that encompass a rapidly growing mass, and the most common
wide spectrum of benign, intermediate, and location is the upper extremity, followed by the
malignant lesions. Fibroblasts are the most com- trunk, head and neck, and lower extremity. This
mon cells in fibrous connective tissue and make tumor occurs in subcutaneous, fascial, or intra-
procollagen, protoelastin, and glycosaminogly- muscular locations. The pathologic diagnosis is
cans. Myofibroblasts are a differentiated cell type made by identifying immature plump fibroblasts
that are essential for wound healing, participating that are similar in size, arranged in short irregular
in tissue remodeling following insults. bundles and fascicles, a feathery appearance,
Myofibroblasts are typically activated fibroblasts, and abundant myxoid changes (Khuu etal. 2014).
although they can also be derived from other cell Imaging findings of nodular fasciitis are vari-
types, including epithelial cells, endothelial cells, able and nonspecific as it manifests diverse histo-
and mononuclear cells (Watsky etal. 2010). An logic subtypes composed of myxoid, cellular, and
important step of forward in the 2013 WHO clas- fibrous components. MR imaging shows homo-
sification of benign fibroblastic/myofibroblastic geneous T1 hypointensity, heterogeneous T2
lesions has been the recognition that nodular fas- hyperintensity, and heterogeneous enhancement.
ciitis, and by analogy the variants proliferative Some lesions demonstrate an inverted target
fasciitis and proliferative myositis, are in fact sign, with a discrete central T2 hyperintensity,
neoplastic (Fletcher 2014). Other notable changes peripheral T2 hypointensity, and a central area
in this category include the complete omission of that does not enhance following contrast admin-
hemangiopericytoma, the prior synonym of istration (Luo etal. 2014). On US, nodular fasci-
extrapleural solitary fibrous tumor (Jo and itis appears as a hypoechoic mass with mixed
Fletcher 2014). echogenicity. A fascial tail may suggest nodu-
lar fasciitis as a potential diagnosis (Khuu etal.
2014).
5.1 Nodular Fasciitis
Nodular fasciitis is a self-limiting reactive pro- 5.2 Proliferative Fasciitis

cess of fibroblastic/myofibroblastic differentia-
tion and the most common benign soft tissue Proliferative fasciitis is characterized by a diffuse
tumor of fibrous origin. The lesion often comes to infiltrative fibroblastic growth that is intimately
clinical attention due to its rapid growth, rich cel- associated with multifocal proliferation of large
lularity, and mitotic activity and can be misdiag- basophilic cells, closely resembling ganglion

64 5 Fibroblastic/Myofibroblastic Tumors
cells. The lesion may be confused with sarcoma 5.4 Myositis Ossificans
because of its rapid growth and its bizarre histo-
logic features; however, the lesion is adequately Historically, the term myositis ossificans (MO)
treated by local excision, and there is no indica- has been used to describe a broad spectrum of
tion for radical surgery (Chung and Enzinger conditions, ranging from benign solitary lesions
1975). to progressive congenital syndromes (Walczak
The imaging appearance of proliferative fasci- etal. 2015). Recently, MO has been defined as a
itis has not been well described. The upper benign, solitary, ossifying soft tissue mass that
extremity is the most common anatomical loca- most commonly occurs in the skeletal muscle.
tion, and most lesions occur in the subcutaneous MO can also be found in other sites, such as the
layer. subcutaneous layer or the fascial planes. MO
often has been used interchangeably with hetero-
topic ossification (HO), which is the formation of
5.3 Proliferative Myositis bone outside the skeletal system. HO is consid-
ered more comprehensive than MO given that the
Proliferative myositis is a rare fibroblastic reac- pathologic process of HO can occur in variable
tive soft tissue lesion and is the intramuscular sites, such as the skin, subcutaneous tissue, skel-
counterpart of proliferative fasciitis. Patients etal muscle, fibrous tissue adjacent to joints,
with proliferative myositis present with a firm, blood vessels, ligaments, and mesentery
rapidly growing mass that can double in size (Mavrogenis etal. 2011).
within several days. Proliferative myositis dif- Three overlapping stages of progression of
fusely involves the muscular tissue, and there is MO have been defined using the following crite-
extensive fibroblast proliferation in the perimy- ria (Kransdorf etal. 1991). Early lesions primar-
sium and epimysium between muscle fascicles. ily consist of a nonossified central core of
Transverse US images may show a character- proliferating benign fibroblasts and myofibro-
istic checkerboard pattern, which has been blasts, with a minor component of osteoid and
described as a thick hypoechoic scaffolding mature lamellar bone at the periphery. Hyaline
that resembles dry, cracked mud (Sarteschi etal. cartilage can be present as part of the endochon-
1997). Longitudinal images reveal the continuity dral calcification. Intermediate lesions have
of muscle fibers. On MR imaging, proliferative either minor or no proliferating fibroblastic core,
myositis appears as an expansile mass of the consisting almost entirely of osteoid rimmed by
muscle, preserving the muscle fascicles without active osteoblasts and surrounded by a shell of
disruption. The lesion appears hypointense or mature lamellar bone. Late lesions consist exclu-
isointense on T1-weighted images and moder- sively of mature lamellar bone.
ately or markedly hyperintense on T2-weighted Radiographs show a soft tissue mass, and the
images. After intravenous injection of the con- calcification may first appear at 3 or 4weeks.
trast agent, the lesion demonstrates prominent Over the next few weeks, flocculent calcifications
enhancement. On axial T2-weighted and post- deposit at the periphery of the mass. These calci-
contrast T1-weighted images, geometrical pat- fications mature during the next several weeks
terns of internal strands of fibroblastic and become coarser and denser. The central zone
proliferation, which are hyperintense compared of the lesion remains lucent or lightly mineral-
to muscle fascicles, are observed within the ized in late (mature) lesions. CT is the best
lesion. This appearance is suggestive of the afore- modality for depicting this zonal phenomenon of
mentioned checkerboard-like pattern. Another calcification in MO.
MR imaging finding of proliferative myositis is MR imaging is the most important imaging
perilesional edema and contrast enhancement study for patients with MO.In early lesions, MO
that extends to the surrounding fascia (Yigit etal. appears as a heterogeneous soft tissue mass with
2009). increased signal intensity on T2-weighted images
5.7 Fibromatosis Colli 65
and is accompanied by extensive, diffuse sur- an echogenic background. However, in some

rounding edema. The margins of early lesions cases, the sonographic pattern of elastofibroma
may be difficult to separate from the surrounding dorsi may be very similar to that of the surround-
edema. When hematoma is present within MO, ing muscular tissue. In these cases, the elastofi-
the area is hyperintense on T1-weighted images broma may be very difficult to distinguish from
and does not enhance after contrast administra- surrounding tissue (Ozpolat etal. 2008).
tion. Curvilinear and irregular areas of decreased
signal intensity can be observed surrounding
intermediate lesions, indicating zonal phenome- 5.6 Fibrous Hamartoma
non. Adjacent soft tissue edema may persist or ofInfancy
disappear at this stage. Late lesions are well-
defined masses with a central fat signal sur- Fibrous hamartoma of infancy (FHI) typically
rounded by a low signal rim. The adjacent soft presents as a solitary, painless, skin-colored subcu-
tissue edema is completely resolved around taneous nodule, which is usually freely movable.
mature lesions. Most lesions occur within the first year of life, and
variable growth rates have been described. This
tumor can become quite large, but the size is usu-
5.5 Elastofibroma ally less than 5cm (Chang etal. 2010). Microscopic
evaluation consistently reveals a mixture of three
Elastofibroma dorsi is a benign fibroelastic tumor tissue types in varying proportions as follows:
of unknown etiology. Histologically, it is com- well-defined bundles of dense fibrous connective
posed primarily of hyalinized collagen with scat- tissue, primitive mesenchyme, and interposed
tered fibroblasts and entrapped islands of mature mature adipose tissue (Stensby etal. 2014).
adipose tissue. In all cases, variable numbers of MR images generally reflect the histological
characteristically enlarged, hypereosinophilic, characteristics of FHI with the three different tis-
refractile, elastic fibrils are present (Kudo 2001). sues. The interspersed fat content of the tumor
Elastofibroma dorsi is usually found in the infra- usually accounts for the high signal intensity on
scapular regions, deep to the serratus anterior and T1-weighted images. Soft tissue strands consisting
latissimus dorsi muscles. Elastofibroma rarely of mesenchymal and fibrous components appear
occurs in other locations, such as the hand, foot, as T1 hypointense, STIR hyperintense, enhancing
greater trochanter, ischial tuberosity, olecranon, tissues. The intervening hypointense septa always
and epidural space. reflect the presence of dense fibrous trabeculae.
Imaging findings of elastofibroma dorsi are
characteristic in its typical location (i.e., in the
infrascapular region). CT and MR imaging show 5.7 Fibromatosis Colli
a crescent-shaped soft tissue mass between the
rib cage and posterolateral chest wall muscles. Fibromatosis colli is an uncommon benign fusi-
CT demonstrates low-attenuation fatty streaks form mass of the sternocleidomastoid, which
within the mass. On T1-weighted MR images, presents as a slow-growing, palpable neck mass.
elastofibroma dorsi appears isointense to the The affected patients are usually within the first
skeletal muscle and may have linear strands of 8weeks of life, and there is a slight male pre-
fat. A relatively low signal intensity (slightly dominance. Most patients present with unilateral
higher than muscles) on T2-weighted images can disease. In approximately 20% of cases, there is
be observed due to dense fibrous connective tis- associated torticollis, causing an ipsilateral tilt of
sue. The degree of enhancement on CT or MR the head and contralateral rotation of the face
imaging is variable, from mild to prominent. andchin. In addition, it is thought that 620%
Elastofibroma has a typical sonographic appear- ofaffected infants will have associated
ance consisting of arrays of linear strands against musculoskeletal abnormalities, such as facial

asymmetry and hip dysplasia (Skelton and the subcutaneous or deep soft tissue. This tumor
Howlett 2014). is composed of spindle- and stellate-shaped
US is the imaging modality of choice for diagno- fibroblasts and abundant interstitial collagen.
sis of fibromatosis colli. It appears as a diffuse or Desmoplastic fibroblastoma should be differenti-
focal enlargement of the sternocleidomastoid and ated from fibromatosis, which has a high risk of
involves the lower two-thirds of the muscle. local recurrence if simple local excision is per-
Fibromatosis colli is typically fusiform in shape and formed. Fibromatosis is more cellular and shows
has variable echogenicity as follows: hyperechoic, short fascicular arrangements of tumor cells and
isoechoic or hypoechoic. CT imaging of fibromato- greater infiltration at the periphery than desmo-
sis colli demonstrates focal or diffuse isodense plastic fibroblastoma (Fukunaga and Ushigome
enlargement of the sternocleidomastoid muscle. On 1999).
MR imaging, there may be a decrease in signal MR imaging shows a well-defined mass with
intensity on T2-weighted images due to the pres- a predominantly low signal intensity on
ence of fibrous tissue; however, the signal character- T2-weighted images. Those areas of marked T2
istics may vary, similar to its echogenicity on US hypointensity do not enhance on gadolinium-
(Ablin etal. 1998; Skelton and Howlett 2014). enhanced images and correspond to a hypocellu-
lar component with dense collagen fibers. The
interspersed areas showing intermediate signal
5.8 Fibroma ofTendon Sheath intensity on T2-weighted images correspond to
hypercellular areas within the lesion that reflect
The designation fibroma of tendon sheath has its loose collagen fibers (Shuto etal. 2002).
been used to describe a group of clinically and Unlike fibromatosis, desmoplastic fibroblasto-
pathologically similar lesions occurring in the mas are clearly circumscribed and do not have
distal aspects of the extremities (Pulitzer etal. areas showing very high signal intensity on
1989). This tumor has a predilection for the flexor T2-weighted images.
aspect of the digits and palm and is frequently
attached to the tendon sheath. The tumor has cir-
cumscribed margins with spindle/stellate cells in 5.10 Calcifying Aponeurotic
a collagenous stroma and slit-like vessels. Many Fibroma
tumors show areas of hypercellularity adjacent to
areas of hypocellularity (Al-Qattan 2014). Calcifying aponeurotic fibroma, also known as
The diagnosis of fibroma of the tendon sheath juvenile aponeurotic fibroma, is a rare benign
is favored when the mass is located in an upper soft tissue tumor with intermediately aggressive
extremity adjacent to a tendon and MR imaging biologic behavior. It is found most commonly
shows a focal nodular mass with decreased signal during the first and second decade of life, and the
on all pulse sequences and little or no enhance- commonly involved sites are the hand, finger and
ment. Fibroma of the tendon sheath, however, foot. This type of fibroma occasionally arises in
can have different imaging features if areas of other sites, including the elbow, ankle, knee, and
increased cellularity or myxoid change are pres- thigh. Most of these tumors are predominantly
ent (Fox etal. 2003). This histologic heterogene- subcutaneous, but some involve the intermuscu-
ity makes the tumor have various signal intensities lar layers. Calcifying aponeurotic fibroma has
and variable enhancement. irregular, ill-defined contours and typically con-
tains the following two components: (1)
fibromatosis- like spindle-shaped cell elements;
5.9 Desmoplastic Fibroblastoma and (2) distinctive, round or oval, fibrocartilagi-
nous, often calcified foci that are composed of
Desmoplastic fibroblastoma, also referred to as epithelioid cells (Murphey etal. 2009).
collagenous fibroma, is a slow-growing benign Radiography may show stippled calcifications
fibroblastic/myofibroblastic tumor that involves and cortical erosion of the adjacent bone. On MR
5.13Dermatofibrosarcoma Protuberans 67
imaging, most of these tumors are ill-defined, lobu- tasis. DF is defined as a benign soft tissue neo-
lated soft tissue masses with adherence to surround- plasm composed of a clonal proliferation of
ing structures. T2-weighted images show fibroblasts and myofibroblasts. In addition, DF
homogeneous or heterogeneous high signal inten- grows with infiltrative margins and is composed
sity. Areas demonstrating low signal intensity on of a proliferation of uniform spindle cells with
T2-weighted image have marked hypocellularity collagen production (Fletcher etal. 2013). This
and abundant collagen (Kwak etal. 2004). tumor can arise anywhere in the body. Its high
Gadolinium-enhanced images show heterogeneous, tendency to recur after surgical resection makes
relatively strong enhancement. Speckled foci of cal- DF an important cause of morbidity and, occa-
cification can also be observed on MR images. sionally, mortality (Braschi-Amirfarzan etal.
2016).
The principal role of imaging in the manage-
5.11 Palmar/Plantar Fibromatosis ment of DF is preoperative planning and the
detection of recurrence or disease progression in
Fibromatosis can be divided into superficial and nonsurgically managed patients (Lee etal. 2006).
deep subtypes. Superficial fibromatoses are usually MR imaging is the modality of choice for evalu-
small (< 5cm) slow-growing lesions that rarely ating abdominal wall and extra-abdominal
involve deep structures. Superficial fibromatosis DF.The lesions may be well defined or infiltra-
include palmar and plantar fibromatosis (Lee etal. tive, and the signal intensity is variable depend-
2006). Patients with palmar fibromatosis present ing on the relative amount of collagen, spindle
clinically with painless, subcutaneous nodules. cells, and extracellular matrix. The MR signal
These nodules may progress slowly to fibrous cords characteristics of DF are a mixed pattern, low to
or bands that attach to and cause traction on the high signal on T2-weighted images and low to
underlying flexor tendons, resulting in flexion con- iso-signal on T1-weighted images. In most cases
tractures of the digits (Dupuytrens contractures). of DF, there are very low T2 signal components
Ulnar-sided rays, particularly the fourth and fifth corresponding to areas of collagen deposition.
digits, are those that are most commonly involved in These regions do not enhance after administra-
palmar fibromatosis (Murphey etal. 2009). In tion of gadolinium-based contrast material. The
patients with palmar fibromatosis, predominantly other, more cellular, portions exhibit moderate-
low signal intensity on T2-weighted images corre- to-marked enhancement. The observation of T2
sponds to relative hypocellularity caused by a mass hypointense, non-enhancing band-like compo-
that predominantly consists of dense collagen. nents increases diagnostic confidence.
Those lesions that demonstrate intermediate signal
intensity on T2-weighted images are more cellular
and thus more likely to locally recur (Robbin etal. 5.13 Dermatofibrosarcoma
2001). Plantar fibromatosis occurs most commonly Protuberans
in the medial aspect of the plantar aponeurosis.
They may appear as single or multiple subcutane- Dermatofibrosarcoma protuberans (DFSP) is a
ous nodules on US and MR imaging. Most plantar low-grade sarcoma that originates in the reticular
fibromatoses demonstrate iso- to high signal inten- dermis of the skin. It most commonly affects
sity on T2-weighted images, while mature lesions young to middle-aged adults, with the tumor pre-
have low signal intensity. dominantly occurring on the trunk, followed by
the extremities and head and neck region. DFSP
typically presents as a firm, skin-colored, indu-
5.12 Desmoid-Type Fibromatosis rated plaque, 15cm in diameter, with one or
more reddish-purple exophytic nodules. Although
Desmoid-type fibromatosis (DF), also known as it superficially tends to spare the epidermis, the
aggressive fibromatosis, is a locally aggressive deep edge classically shows infiltration along
fibroblastic neoplasm with no potential for metas- fibrous septa into underlying subcutaneous fat,
encircling adipocytes and giving rise to a distinc- tissue has low T2 signal intensity. Occasionally,
tive honeycomb pattern (Kuzel etal. 2015). tumors can have focal or diffuse myxoid stroma,
DFSPs are nodular soft tissue mass involving which is apparent as a hyperintense T2 signal.
the skin and subcutaneous tissue on MR imaging Malignant areas within tumors also tend to
with nonspecific signal intensity and enhance- showintermediate-to-high signal intensity on
ment pattern. Although most cases of the tumor T2-weighted images, corresponding to edema
are superficial and well-defined, some may be and increased vascularity. Histopathological cor-
found in a deep location, and rare cases are poorly relation studies have demonstrated that hemor-
defined in appearance (Torreggiani etal. 2002). rhage, cystic degeneration, and necrosis are rare,
MR imaging is superior to clinical palpation in occurring in fewer than 5% of cases. However,
detecting the depth of infiltration of DFSP.For these findings can be observed in larger and
DFSPs in which the tumor has been excised with malignant tumors (Musyoki etal. 2012). The
positive margins, however, MR imaging is not radiological features that correlate better with
useful for determining the persistence of the malignant criteria are tumor size, heterogeneous
tumor. MR imaging does not appear to be useful signal intensity, and heterogeneous uptake of
for determining the lateral limits of DFSP (Serra- contrast on MR imaging (Garcia-Bennett etal.
Guillen etal. 2011). 2012). SFTs tend to displace adjacent structures
but occasionally invade them.
5.14 Solitary Fibrous Tumor

5.15 Inflammatory
Solitary fibrous tumor (SFT) shows a wide spec- Myofibroblastic Tumor
trum of histological features, ranging from het-
erogeneous, multinodular, partially sclerotic Myofibroblasts are one of the main cell types in
lesions to monotonous, highly cellular lesions. connective tissue that share ultrastructural fea-
There are two main subtypes of SFT, one being tures of both fibroblasts and smooth muscle cells.
fibrous (SFT of the pleura). The other is a cellular Myofibroblastic neoplasms encompass a range of
form, which characteristically contains numerous benign to malignant disease. Among malignant
thin-walled vessels with little intervening fibro- tumors, only low-grade forms are currently
sis; these tumors are virtually indistinguishable accepted as a diagnostic category. Inflammatory
from conventional hemangiopericytoma (Gengler myofibroblastic tumor (IMT) and low-grade
and Guillou 2006). Solitary fibrous tumor shows myofibroblastic sarcoma are important interme-
no gender predilection, and most patients are in diate- or low-grade malignant neoplasms in this
the fifth to seventh decades. The pleura is the spectrum (Qiu etal. 2008). IMT is defined as a
most common site of occurrence and the orbits, disease that primarily consists of myofibroblastic
and the extremities (muscles and subcutaneous spindle cells, with variable infiltration of plasma
layer) are the most common extrapleural sites. cells and/or lymphocytes (Tan etal. 2016). IMT
On US, SFT is a well-demarcated mass with is more common in children and young adults
homogeneous or heterogeneous hypoechoic pat- and occurs at a median age of 9years. IMT can
tern. The power Doppler intratumoral signal be found anywhere in the body, and the common
shows either a peripheral or a diffuse pattern, and sites include the lung, soft tissue, and viscera.
a vascular pedicle is commonly observed. On The imaging features of IMT are variable and
MR imaging, the extrapleural SFT is observed as nonspecific. Contrast-enhanced CT may show a
a multilobulating mass in the muscle or subcuta- homogeneous or heterogeneous enhancement of
neous layer, with nonspecific signal intensities the lesion. These tumors typically manifest as
and strong enhancement. T2-weighted imaging lobulated heterogeneous masses, which may
characteristics are helpful in identifying fibrous have calcifications. Large lesions can have non-
content of tumors because dense mature fibrous enhancing central necrotic areas and calcifica-
5.19Sclerosing Epithelioid Fibrosarcoma 69
tions (Sargar etal. 2016). On MR imaging, IMTs LGFMS typically presents during young or mid-
are isointense to muscle on T1-weighted images dle adult life, but there have been an increasing
and hypo- to hyperintense on T2-weighted number of reports of this tumor in the pediatric
images, depending on the relative quantity of age group. LGFMS are observed most frequently
fibrous tissue, cellular material, and necrosis. A on the deep soft tissue of the lower extremity or
dark signal on T2-weighted images is more likely thoracic wall, followed by the shoulder and
dense fibrosis. Heterogeneous and variably inguinal area. Most lesions arise in the deep soft
strong contrast enhancement is observed on post- tissue, but subcutaneous masses can be found in
contrast MR images. children. On histology, LGFMS shows two dis-
tinct zones (myxoid and fibrous), bland regular
spindle cells, and a swirling or whorled pattern,
5.16 Myxofibrosarcoma with absent or sparse mitotic activity.
On MR imaging, LGFMS appears heteroge-
Myxofibrosarcoma (MFS) is a soft tissue sar- neous due to the presence of two distinct internal
coma that typically presents on the extremities of zones (myxoid and fibrous). LGFMS shows areas
elderly patients. It has formerly been referred to of low signal intensity on both T1- and
as myxoid malignant fibrous histiocytoma. The T2-weighted images due to the fibrous compo-
histology of the tumors reveals haphazardly nent (Sargar etal. 2015). On fluid-sensitive
arranged fascicles of atypical spindle-shaped sequences, a distinct gyriform pattern with mul-
cells, with a varying amount of myxoid matrix. tiple folded layers of predominantly low to iso-
Nuclear atypia is variable. A curvilinear vascular signal intensity areas that mimic brain gyri has
network is well developed (Kaya etal. 2008). been reported (Hwang etal. 2012).
Whereas most soft tissue sarcomas grow as
discrete round or oval masses, MFS often
exhibit an infiltrative margin that extends into 5.18 Low-Grade Myofibroblastic
surrounding tissues for substantial distances Sarcoma
along normal anatomical planes, particularly
fascial planes, resulting in distant microscopic Low-grade myofibroblastic sarcoma occurs pri-
tumor deposits that predispose to local recur- marily in adult patients but has been described in
rence after resection. On MR imaging, this infil- patients between 7 and 85years of age. Local
trative spread may manifest as curvilinear recurrences are common, but distant metastases
projections that extend from the primary mass- are infrequent (San Miguel etal. 2004). These
like portion of the MFS (Lefkowitz etal. 2013). tumors have been found in the oral cavity, limbs,
Gadolinium-enhanced MR images depict this abdominal/pelvic cavity, and chest wall. Imaging
infiltrative tumor extension more accurately findings of low-grade myofibroblastic sarcoma
than T2-weighted images. These MR imaging are not well described. The tumors in the somatic
appearances and the tail sign may indicate tumor soft tissue are located in the muscle, perifascial
infiltration along the fascial plane and may also tissue, or subcutaneous layer.
be associated with worse local recurrence-free
survival (Yoo etal. 2014).
5.19 Sclerosing Epithelioid
Fibrosarcoma
5.17 Low-Grade Fibromyxoid
Sarcoma Sclerosing epithelioid fibrosarcoma (SEF) is a
rare malignant fibroblastic tumor and a unique
Low-grade fibromyxoid sarcoma (LGFMS) is a variant of fibrosarcoma. SEF is characterized by
rare spindle cell tumor exhibiting bland histolog- epithelioid cells that are arranged in nests and
ical features but aggressive clinical behavior. strands in a densely hyalinized collagenous
matrix. SEF often occurs in deep soft tissue of images has been reported as a distinctive feature
the lower extremities, limb girdles, trunk, upper (Christensen etal. 1997).
extremities, and the head and neck area (Yoon
etal. 2012). Most of the lesion is heteroge-
neously isointense to muscle on T1-weighted 5.20 Illustrations: Fibroblastic/
images and hyperintense on T2-weighted Myofibroblastic Tumors
images. A central stellate core of very low sig-
nal intensity on both T1- and T2-weighted 5.20.1 Nodular Fasciitis
a b
Fig. 5.1 Nodular fasciitis. Axial T1WI (a) shows a peri- enhancing mass with mild heterogeneity. Tail-like T2
fascial mass on the left medial knee. Axial PDWI (b) and hyperintensity or enhancement (arrows) extends along the
coronal postcontrast FS T1WI (c) show a hyperintense or fascia
5.20Illustrations: Fibroblastic/Myofibroblastic Tumors 71
a b
Fig. 5.2 Nodular fasciitis. Axial T1WI (a) shows a nodu- hyperintense nature of the mass, and axial postcontrast FS
lar subcutaneous mass (arrow) with an infiltrative margin T1WI (c) show intense enhancement. The mass abuts the
in the right proximal thigh. Axial FS T2WI (b) shows the deep fascia over the vastus lateralis
a b
c d
Fig. 5.3 Nodular fasciitis. Axial T1WI (a) shows a central hyperintensity. Axial postcontrast FS T1WI (c)
slightly hyperintense mass (arrow) between the left bra- shows heterogeneous enhancement. On longitudinal US
chial artery and brachialis muscle. Axial T2WI (b) shows (d), the mass has medium-level echogenicity with central
heterogeneous signal intensity of the mass (arrow) with hypoechoic area
5.20.2 Proliferative Fasciitis
a b
c d
Fig. 5.4 Proliferative fasciitis. Axial T1WI (a), FS T2WI enhancement. There are edema-like signals and enhance-
(b), and postcontrast FS T1WI (c) show a perifascial mass ment along the fascia (arrowheads). Longitudinal US (d)
(arrow) on the radial side of the left proximal forearm. shows a hypoechoic mass involving the deep fascia
The mass appears with T2 hyperintensity and prominent (arrow) and subcutaneous layer
5.20.3 Proliferative Myositis
a b
c d
Fig. 5.5 Proliferative myositis. Coronal FS (a) and axial like signal in the surrounding muscle and adjacent subcu-
(b) T2WIs show fusiform hypertrophy of the left sartorius taneous layer. Axial T1WI (c) shows isointensity and axial
muscle, with a poorly demarcated mass (arrow). The postcontrast FS T1WI (d) shows heterogeneous
intralesional muscle fascicles are preserved and separated enhancement
by hyperintense perimysia. There is prominent edema-
a b
Fig. 5.6 Proliferative myositis. Axial T2WI (a) shows a US (b) shows checkerboard appearance consisting of
hyperintense mass (arrow) in the left vastus lateralis mus- hypertrophied muscle fascicles and thickened hypoechoic
cle. There are band-like hyperintensities in keeping with perimysia. Longitudinal Doppler US (c) shows increased
fibroblastic proliferation in the perimysium. Transverse vascularity
5.20.4 Myositis Ossificans
a b
c d
Fig. 5.7 Myositis ossificans. Coronal and axial FS T2WI T1WI (e) shows a well-demarcated mature lesion with a
(a, b) show a hyperintense intramuscular mass surrounded central fat signal (arrows). Axial T2WI (f) shows com-
by intermediate signal rind (arrow). There is an extensive plete resolution of the surrounding soft tissue edema
muscle edema in the left vastus intermedius. The mass is around the mature lesion. Lateral radiograph (g) of the left
iso- to slightly hypointense to muscle on T1WI (c). Axial thigh, which was taken at the same time as the images in
postcontrast FS T1WI (d) reveals a lobulated mass with e and f, shows mature bone formation
prominent peripheral enhancement. After 6months, axial
e g
Fig. 5.7(continued)
a b
Fig. 5.8 Myositis ossificans. Sagittal T2WI (a) shows a postcontrast FS T1WI (c) shows the contrast-avid mass,
hyperintense mass (arrow) surrounded by a low signal rim with prominent enhancement in the surrounding soft
in the left popliteal fossa. Axial FS T2WI (b) shows exten- tissue
sive soft tissue edema (arrows) around the mass. Axial
a c
Fig. 5.9 Myositis ossificans. (a) One week after onset, eralization pattern (arrow) at the periphery of the mass.
axial STIR shows an intramuscular mass in the right (c) Four weeks after onset, AP radiograph of the right hip
adductor magnus, with surrounding edema. (b) Two shows a medial thigh mass with faint mineralization
weeks after onset, axial CT demonstrates the typical min- (arrows)
5.20.5 Elastofibroma Dorsi
a b
Fig. 5.10 Elastofibroma dorsi. Coronal and axial T1WIs T1WI (d) shows heterogeneous enhancement of the mass
(a, b) show a soft tissue mass (arrow) with interspersed (arrow). Longitudinal extended field of view US (e)
lines of high signal between the right rib cage and serratus shows an intratumoral, multilayered pattern of hypoechoic
anterior. Axial T2WI (c) shows the mass (arrow) with a linear areas of fat deposition intermixed with echogenic
mixed intermediate and high signal. Axial postcontrast FS fibroelastic tissue
Fig. 5.11 Elastofibroma dorsi. Axial T1WI (a) show

a
bilateral soft tissue masses (arrows) in the infrascapu-
lar regions. (b) Gross specimen demonstrating patterns
of alternating bands of dense fibrous and fat tissue
5.20.6 Fibrous Hamartoma ofInfancy
a b
Fig. 5.12 Fibrous hamartoma of infancy. Coronal T1WI of the nonadipose components. Longitudinal US (d)
(a) and axial T2WI (b) show a subcutaneous mass (arrow) shows a poorly demarcated hyperechoic mass (arrows)
in the left buttock. The mass has high signal fat compo- with intervening hypoechoic portions. (e) Cut section
nent and low signal mesenchymal and fibrous compo- showing a gray-white tumor with fibrous tissue
nents. Axial postcontrast FS T1WI (c) shows enhancement
c d
a b
Fig. 5.13 Fibrous hamartoma of infancy. Sagittal T1WI and T2WI (a, b) show a poorly circumscribed subcutaneous
mass (arrows) with interspersed fat and soft tissue strands at the sacral level
5.20.7 Fibromatosis Colli
a a
Fig. 5.15 Fibromatosis colli. Transverse US (a) shows a

hyperechoic mass (arrowheads) of the left sternocleido-
Fig. 5.14Fibromatosis colli. Transverse US (a) of the mastoid muscle. Longitudinal US (b) shows a mass-
anterior neck shows asymmetrical thickening of the left forming lesion (arrowheads) of the left sternocleidomastoid
sternocleidomastoid muscle (star). Longitudinal scan (b) without extramuscular involvement
shows fusiform enlargement of the left sternocleidomastoid
(arrowheads) Longitudinal scan (c) shows a normal appear-
ance of the right sternocleidomastoid (arrowheads)
5.20.8 Fibroma ofTendon Sheath
a b
c d
Fig. 5.16 Fibroma of tendon sheath. Sagittal and axial T1WI (c) shows the mass encasing the second extensor
T2WIs (a, b) show a nodular subcutaneous mass (arrow) digitorum longus tendon (arrowhead). Axial postcontrast
over the left second MTP.A heterogeneous, but predomi- FS T1WI (d) shows heterogeneous enhancement of the
nantly hypointense signal is observed on T2WIs. Axial tumor
a b
Fig. 5.17 Fibroma of tendon sheath. Coronal and axial marked hypointensity of the mass (arrow). Axial postcon-
T1WIs (a, b) show a nodular mass (arrows) in the right trast FS T1WI (d) shows a large area of non-enhancement
fourth intermetacarpal space. Axial T2WI (c) reveals at the center of the mass
a b
Fig. 5.18 Fibroma of tendon sheath. Sagittal T1WI (a) tense rind. Sagittal postcontrast FS T1WI (c) shows het-
shows a lobulated mass (arrow) attached to the posterior erogeneous enhancement of the mass (arrow), an effect
knee joint capsule. Sagittal T2WI (b) shows the mass that is more prominent at the periphery
(arrow) with intermediate signal intensity and hypoin-
5.20.9 Desmoplastic Fibroblastoma
a b
c d
Fig. 5.19 Desmoplastic fibroblastoma. Axial T1WI and intensity. Sagittal postcontrast FS T1WI (c) shows mild,
T2WI (a, b) show a large intermuscular mass in the right heterogeneous enhancement. (d) Gross specimen demon-
lower leg. The mass is observed with marked low signal strating fibrous tumor with some myxoid changes
a b
Fig. 5.20 Desmoplastic fibroblastoma. Axial T1WI (a) but predominantly hypointense to muscle. Axial postcon-
shows a well-demarcated soft tissue mass with low to iso- trast FS T1WI (c) shows minimal enhancement through-
signal intensity in the left lateral shoulder. Axial FS T2WI out the mass
(b) shows the mass with a heterogeneous signal intensity
5.20.10 Calcifying Aponeurotic

Fibroma
a b
Fig. 5.21 Calcifying aponeurotic fibroma. Axial T1WI led foci of calcification (arrow) of low signal intensity.
(a) shows a soft tissue mass over the second metacarpal Axial postcontrast FS T1WI (c) shows heterogeneous
base. There is a bone erosion (arrow) at the dorsal side of enhancement of the mass
second metacarpal base. Axial FS T2WI (b) shows speck-
a b
Fig. 5.22 Calcifying aponeurotic fibroma. Axial T1WI appearance in the central hypointense area. Sagittal post-
(a) shows a soft tissue mass (arrow) at the plantar side of contrast FS T1WI (c) shows prominent enhancement of
the left second toe proximal phalanx. The second flexor the mass (arrow). Transverse US (d) shows a lobulated
digitorum longus tendon is partially encased by the mass. hypoechoic mass (arrowheads) with punctate hyper-
Axial T2WI (b) shows central low and peripheral high echoic foci
signal intensity of the mass. The mass has a fascicular
c d
5.20.11 Palmar/Plantar Fibromatosis
a b
c d
Fig. 5.23 Plantar fibromatosis. Longitudinal US (a) of attached to the plantar fascia (arrow). Sagittal FS T2WI
the sole reveals a soft tissue mass (arrows) arising within (c) shows the hyperintense mass (arrow) with scattered
the plantar fascia (arrowheads) on the plantar side of the low signal components. Sagittal postcontrast FS T1WI (d)
right first metatarsal. The mass has an irregular margin shows prominent enhancement of the mass (arrow).
and heterogeneous echotexture. Axial T1WI (b) shows an Another small enhancing lesion (arrowhead) is observed
iso-signal intensity mass (star) with low signal foci in the plantar fascia proximal to the main mass
5.20.12 Desmoid-Type Fibromatosis
a b
c d
Fig. 5.24 Desmoid-type fibrosis. Axial T1WI (a) shows T1WI (c) shows prominent enhancement of the mass,
a deep soft tissue mass (arrow) that is isointense to muscle which has an infiltrative margin (arrows) with the adja-
around the left scapula inferior angle. On axial T2WI (b), cent muscle and subcutaneous fat. (d) Cut section show-
the mass is hyperintense and has some band-like low sig- ing infiltrative margins (arrowheads) of the tumor with
nal components (arrowheads). Axial postcontrast FS the subcutaneous fat and muscle
a b
Fig. 5.25 Desmoid-type fibrosis. Axial T1WI (a) shows portion with a very low signal intensity (star). Axial post-
an intermuscular mass (arrows) in the posterior compart- contrast FS T1WI (c) shows prominent enhancement of
ment of the right thigh. The mass is isointense to muscle the mass, except the densely fibrotic area (star). The pos-
and markedly hypointense at the center (star). Axial terior margin of the tumor is infiltrative and extends into
T2WI (b) shows a mass with a high signal intensity and a the semitendinosus muscle (arrows)
a b
Fig. 5.26 Desmoid-type fibrosis. Axial T1WI (a) shows with predominantly low signal intensity (arrows). Axial
a poorly defined soft tissue mass around the left second postcontrast FS T1WI (c) shows interspersed enhancing
metatarsal. Axial T2WI (b) shows the infiltrative mass and non-enhancing parts of the mass
a b
Fig. 5.27 Desmoid tumor of the abdominal wall. Axial several small areas of low signal intensity. Coronal post-
T1WI (a) shows a soft tissue mass (arrow) in the left rec- contrast FS T1WI (c) shows prominent enhancement of
tus abdominis; the mass is largely isointense to muscle. the tumor, with an irregular non-enhancing segment in the
Axial T2WI (b) shows the relatively well-demarcated distal portion (arrow)
mass (arrow) to be slightly hyperintense to muscle, with
5.20.13 Dermatofibrosarcoma
Protuberans
a b
Fig. 5.28Dermatofibrosarcoma protuberans. Axial The mass is slightly hyperintense to muscle, with several
T1WI (a) shows an exophytic mass with slight hyperin- small areas of lower signal intensity. Coronal postcontrast
tensity in the right lateral proximal upper arm. Axial FS T1WI (c) clearly reveals a small satellite lesion (arrow)
T2WI (b) shows the hyperintense subcutaneous mass, on the anterior side of the main tumor
which slightly compresses the subjacent deltoid muscle.
a b
Fig. 5.29Dermatofibrosarcoma protuberans. Axial hyperintensity. Coronal postcontrast FS T1WI (c) shows
T1WI (a) shows a subcutaneous mass (arrows) that abuts strong enhancement of the lesion, with a central non-
the skin in the left lateral proximal upper arm. Axial enhancing portion
T2WI (b) shows the mass (arrows) with heterogeneous
5.20.14 Solitary Fibrous Tumor
a b
Fig. 5.30 Solitary fibrous tumor. Axial T1WI (a) shows sue. Axial postcontrast FS T1WI (c) shows marked
an isointense subcutaneous mass (arrow) in the left but- enhancement of the lesion and several non-enhancing
tock. Axial T2WI (b) shows a hyperintense mass with fibrotic components
scattered low signal foci, suggestive of dense fibrous tis-
a b
c d
Fig. 5.31 Solitary fibrous tumor. Axial T1WI (a) shows neous enhancement of the lesion. There are multiple
an intermuscular mass between the sartorius and the intratumoral serpentine vessels with signal voids (arrows
adductor longus in the left thigh. The mass appears rather in b, c). Contrast-enhanced CT scan (d) shows the
heterogeneous, iso- to hyperintense to muscle. Axial intensely enhancing mass with some non-enhancing
T2WI (b) shows the mass with heterogeneous hyperinten- areas. Color Doppler US (e) demonstrates prominent vas-
sity. Axial postcontrast FS T1WI (c) shows a homoge- cularity within the tumor
5.20.15 Inflammatory
Myofibroblastic Tumor
a c
Fig. 5.32 Inflammatory myofibroblastic tumor. Axial hyper- and hypointense mass (arrow) with dark compo-
T1WI (a) shows an intramuscular mass (arrow) in the nents. Coronal postcontrast FS T1WI (c) shows a lobu-
right gluteus maximus. Axial T2WI (b) shows a mixed lated mass with prominent enhancement
a b
Fig. 5.33 Inflammatory myofibroblastic tumor. Axial ately hyperintense relative to the muscle. Transverse US
T1WI (a) and T2WI (b) show a subfascial mass (arrows) (c) shows a well-demarcated hypoechoic mass with fine
in the right anterior deltoid muscle. The mass is moder- internal echogenicity
5.20.16 Myxofibrosarcoma
a b
Fig. 5.34 Myxofibrosarcoma. Axial T1WI (a) shows a trast FS T1WI (c) shows prominent and heterogeneous
subcutaneous mass in the left lateral thigh. Axial FS T2WI enhancement of the mass. The tumor has a tail-like exten-
(b) shows a mass with predominantly myxoid tissue and sion (arrows in b, c) along the fascial plane
multiple hypointense linear fibrous septa. Axial postcon-
a b
Fig. 5.35 Myxofibrosarcoma. Axial T1WI (a) shows a tense components. Axial postcontrast FS T1WI (c) shows
subcutaneous mass in the left distal anterior thigh. Axial strong enhancement of the mass and clearly demonstrates
T2WI (b) shows a hyperintense mass with some hypoin- an enhancing tail-like structure (arrow)
a b
Fig. 5.36 Myxofibrosarcoma. Axial T1WI (a) shows an are tail-like enhancing lesions along the fascia (arrows)
intermuscular mass with iso- and low signal intensity in and fuzzy enhancement in the adjacent vastus lateralis
the right lateral thigh. Axial T2WI (b) shows a heteroge- muscle (thick arrow). (d) Gross specimen showing myx-
neously hyperintense mass. Axial postcontrast FS T1WI oid stroma with areas of hemorrhage and necrosis
(c) shows heterogeneous enhancement of the mass. There
c d
5.20.17 Low-Grade Fibromyxoid

Sarcoma
a b
Fig. 5.37Low-grade fibromyxoid sarcoma. Coronal Axial T1WI (b) shows an isointense mass (arrow). Axial
T2WI (a) shows an intermuscular mass in the left proxi- postcontrast FS T1WI (c) shows heterogeneous enhance-
mal thigh. There is a gyriform pattern (arrows) that con- ment of the mass, which exhibits a myxoid pattern
sists of multiple folds with relatively low signal intensity.
a b
Fig. 5.38 Low-grade fibromyxoid sarcoma. Axial T1-, T2-, and postcontrast FS T1WI (ac) show a subfascial mass
(arrow) in the right posterolateral lower leg. The mass involves the deep fascia and extends to the subcutaneous layer
a b
Fig. 5.39Low-grade fibromyxoid sarcoma. Coronal band-like low signal components (arrows) correspond to
T1WI (a) shows an intramuscular mass in the left rectus fibrous tissue. Coronal postcontrast FS T1WI (c) shows
femoris. Coronal T2WI (b) reveals a T2 hyperintense prominent enhancement of the largely myxoid tumor
mass with a predominantly myxoid appearance. Multiple
5.20.18 Low-Grade Myofibroblastic

Sarcoma
a b
Fig. 5.40 Low-grade myofibroblastic sarcoma. Sagittal exhibiting a fluid-like signal. Axial postcontrast FS T1WI
T1WI (a) shows an intermuscular mass (arrow) between (c) shows prominent enhancement at the peripheral por-
the rhomboid major and trapezius. Sagittal FS T2WI (b) tion of the tumor
shows a hyperintense mass with a large central area (star)
a b
Fig. 5.41 Low-grade myofibroblastic sarcoma. Axial neously hyperintense mass (arrow) intermingled with
T1WI (a) shows an isointense mass (arrow) in the right muscle fascicles. Axial postcontrast FS T1WI (c) shows
iliopsoas muscle. Axial FS T2WI (b) shows a heteroge- heterogeneous enhancement of the mass (arrow)
5.20.19 Sclerosing Epithelioid

Fibrosarcoma
a b
Fig. 5.42Sclerosing epithelioid fibrosarcoma. Axial keeping with dense fibrosis. Axial and coronal postcon-
T2WI (a) reveals a large axillary mass with a relative T2 trast FS T1WI (b, c) shows heterogeneous enhancement
hypointensity. There are areas of central fluid-like signal of the mass, which exhibits central non-enhancing areas
(arrow) and marked T2 hypointensity (thick arrow), in (arrows)
Chung EB, Enzinger FM.Proliferative fasciitis. Cancer.

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So-Called Fibrohistiocytic Tumors
6
Using electron microscopy, immunohistochemis- mononuclear cells, accompanied by a variable

try, and cytogenetics, it has come to be under- number of multinucleate osteoclast-like cells,
stood that most tumors that were initially foamy histiocytes (xanthoma cells with choles-
considered to be various types of fibrohistiocytic terol clefts), siderophages (hemosiderin deposit),
tumors are actually not histiocytic in origin. and inflammatory cells. The diffuse type of
Rather, these tumors are a diverse group of neo- TSGCT is referred to as diffuse-type giant cell
plasms that only appear histologically similar to tumor, pigmented villonodular synovitis, or pig-
fibrohistiocytic tumors. In the revised 2013 WHO mented villonodular tenosynovitis. This tumor is
classification of soft tissue sarcomas, the term a locally aggressive neoplasm but is composed of
malignant fibrous histiocytoma was removed similar cells as in the localized type (de Saint
from the so-called fibrohistiocytic tumor cate- Aubain Somerhausen and van de Rijn 2013).
gory. The so-called fibrohistiocytic tumors are The localized type of TSGCT is the most
composed of the following four tumors: tenosy- common type of giant cell tumors. These tumors
novial giant cell tumor, deep benign fibrous his- usually occur in patients between 30 and 50years
tiocytoma, plexiform fibrohistiocytic tumor, and of age and exhibit a 2:1 female predominance.
giant cell tumor of soft tissue. This lesion occurs predominantly in the hand and
is the second most common benign tumor of the
hand after ganglion cysts. The diffuse type of
6.1 enosynovial Giant Cell
T TSGCT tends to affect younger patients
Tumor (aged<40years) than the localized type. Diffuse
type TSGCTs affect large joints, predominantly
Tenosynovial giant cell tumors (TSGCT) encom- the knee, followed by the hip, ankle, elbow, and
pass a family of lesions that arise from the shoulder. The localized type of TSGCT often
synovium of joints, bursae, or tendon sheath, presents with a firm, slow-growing, non-tender
which show synoviocytic differentiation (Boland mass. Patients with the diffuse type complain of
etal. 2009). These tumors are divided into intra- pain, tenderness, swelling, or limitation of motion
or extra-articular types according to their loca- (de Saint Aubain Somerhausen and van de Rijn
tion and into localized or diffuse types according 2013; Ushijima etal. 1986).
to their growth pattern. The localized type of Radiographic appearances vary by the type
TSGCT is also known as giant cell tumor of ten- and size of the lesions. Localized extra-articular
don sheath or nodular tenosynovitis. This is a lesions appear as soft tissue masses, with occa-
benign neoplasm composed of synovial-like sional pressure erosion of the adjacent bone.

110 6 So-Called Fibrohistiocytic Tumors
Thediffuse intra-articular lesions commonly 6.2 eep Benign Fibrous

D
manifest as joint effusion, soft tissue swelling, Histiocytoma
absence of mineralization, extrinsic erosions of
bone, preservation of joint space, and normal Benign fibrous histiocytoma (BFH) is among the
bone mineralization (Murphey etal. 2008). On most common soft tissue skin tumor and is com-
US, TSGCTs of the localized type may appear as posed of bland fibroblasts arranged in a storiform
a well- circumscribed, solid, homogeneous, or pattern with variable giant cells and foamy histio-
heterogeneous hypoechoic soft tissue mass close cytes (Gleason and Fletcher 2008; Fletcher
to the tendon or joint, with detectable internal 1990). BFH is classified into cutaneous and deep
vascularity on color or power Doppler US types. Deep BFH is very rare, comprising fewer
(Middleton etal. 2004). MR imaging is the than 5% of all BFHs, and arises in subcutaneous
modality of choice for diagnosis of or deep soft tissue. This type is commonly found
TSGCT.TSGCT typically displays a low-to- in the lower extremities, followed by the head
intermediate signal on T1-weighted images. and neck region. It develops at any age and show
Intralesional hypointense foci on T2-weighted a slight predominance in males (Fletcher 1990).
images due to the paramagnetic effect of hemo- The radiologic findings for this type are not
siderin are characteristic of TSGCT.On gradient- specific. It is reported that US reveals a well-
echo images, the paramagnetic effect of defined hyperechoic soft tissue mass with occa-
hemosiderin (blooming effect) is further exag- sional hypervascularity on Doppler images.
gerated, creating areas of very low signal inten- CTand MR imaging demonstrate a well-
sity. MR imaging effects of hemosiderin vary circumscribed, smoothly marginated soft tissue
more in the localized type of TSGCT.In cases mass in the subcutaneous fat or deep soft tissue,
with more limited hemosiderin deposits, signal with variable contrast enhancement depending
intensity may be hyperintense on T2-weighted on the vascularity, intralesional hemorrhage, and
images than expected. Diffuse contrast enhance- necrosis (Thomas etal. 2015; Jo etal. 2015;
ment of varying degree is observed in most cases Chung etal. 2011; Machiels etal. 1998). When
(Al-Nakshabandi etal. 2004; Murphey etal. the lesion is large or necrotic, one must differen-
2008). Additional MR imaging features of diffuse tiate deep BFH from superficial soft tissue sarco-
intra-articular TSGCT include heterogeneous, mas, such as undifferentiated pleomorphic
diffuse, plaque-like, or nodular synovial thicken- sarcoma, cutaneous leiomyosarcoma, and derma-
ing, bone erosion or subchondral cysts, septation, tofibrosarcoma protuberans (Thomas etal. 2015).
edema in the adjacent bone or soft tissue, and The optimal treatment for BFH includes a wide
articular cartilage defects (Hughes etal. 1995). excision and continuous follow-up given that local
The recurrence rate for the localized type is recurrence or metastasis can occur in deep fibrous
generally lower than for the diffuse type. The histiocytomas (Gleason and Fletcher 2008).
recurrence following surgical resection of the
localized type of TSGCT ranges from 0% to
44%; however, these recurrences are usually non- 6.3 iant Cell Tumor ofSoft
G
destructive and controllable by surgical re- Tissue
excision. Factors that have been suggested to
increase the recurrence rate include lesion loca- Giant cell tumor of soft tissue (GCT-ST) is a rare
tion, history of previous operations, and positive primary soft tissue neoplasm that clinically and
surgical margins (Murphey etal. 2008; Schwartz morphologically resembles a giant cell tumor
etal. 1989; Flandry and Hughston 1987). ofbone. GCT-ST displays a multinodular archi-
Therefore, diffuse type of TSGCT is regarded as tecture, with nodules composed of a mixture
a locally aggressive but non-metastasizing neo- ofround to oval mononuclear cells and
plasm, and wide excision is the treatment of osteoclast-like giant cells immersed in a richly
choice (de Saint Aubain Somerhausen and van de vascularized stroma (Oliveira 2013). Additional
Rijn 2013). histologic features include blood-filled cystic
spaces similar to those within aneurysmal bone As GCT-ST is rare, there have been only a
cysts, metaplastic bone formation in the lesion small number of reports regarding imaging find-
periphery, stromal hemorrhage, marked stromal ings for this lesion. US of GCT-ST reveals a well-
fibrosis, and clusters of foamy macrophages demarcated, heterogeneous hypoechoic solid
(Oliveira 2013; OConnell etal. 2000). GCT-ST mass with intralesional vascularity on color
is associated with a local recurrence rate of 12% Doppler images. On MR imaging, this tumor
and very rare cases of metastases or death. shows hyperintense signal relative to muscle on
GCT-ST is regarded as an intermediate malig- T2-weighted images and intermediate to hypoin-
nancy (rarely metastasizing) according to the tense signal on T1-weighted images, with intense
2013 WHO classification of soft tissue tumors. contrast enhancement. Hemorrhagic cystic
Therefore, complete removal of GCT-ST with changes with fluid-fluid levels have been occa-
negative surgical margin is advised. sionally reported, similar to aneurysmal bone
GCT-ST usually presents as a well- cysts (Meana Moris etal. 2010; An etal. 2008).
circumscribed, slow-growing mass, which usu-
ally occurs in middle-aged to older individuals,
although age at presentation varies. There is no 6.4 Illustrations: So-Called
sex predilection. This lesion occurs most com- Fibrohistiocytic Tumors
monly in the lower limbs, followed by the trunk
and upper limbs. It usually involves the superfi- 6.4.1 Tenosynovial Giant Cell
cial soft tissue but can occur in deep soft tissue Tumor, Localized Type
(Oliveira etal. 2000; OConnell etal. 2000).
a b
Fig. 6.1Tenosynovial giant cell tumor, localized type. addition, some thick hypointense lines (arrows) represent
Coronal T1WI (a) shows a multilobulated mass (star) that is fibrous septae with hemosiderin deposits. Sagittal postcon-
located within the intermuscular space of the popliteal fossa trast FS T1WI (c) shows the mass to have heterogeneous
of the knee. This mass is slightly hyperintense relative to intense enhancement. There is no synovial hypertrophy or
skeletal muscle. Note multifocal nodular or curvilinear dark abnormal enhancement of the knee. US (d) reveals a well-
foci (arrowheads), in keeping with hemosiderin deposits. On marginated solid mass in the deep popliteal fossa (star) with
sagittal T2WI (b), the mass is intimately associated with the a heterogeneous echo pattern, ranging from small hypoechoic
anterior cruciate ligament and posterior joint capsule of the foci to regions that are isoechoic to hyperechoic relative to
knee. The mass has an inhomogeneous low to intermediate subcutaneous fat. A photograph of the specimen (e) shows a
hyperintensity relative to skeletal muscle. Small dark foci well-circumscribed, multilobulated, yellowish mass with

(hemosiderin deposits) throughout the lesion are seen. In multifocal brown areas (arrowheads)
c d
Fig. 6.1(continued)
a b
Fig. 6.2 Tenosynovial giant cell tumor, localized type. neously enhanced on postcontrast FS T1WI (d).
Coronal T1WI (a) reveals a circumscribed intra-articular Hypointense foci (small arrows) on all sequences repre-
hyperintense (relative to muscle) soft tissue mass (arrow) sent hemosiderin deposits. There is a moderate amount of
in the left hip joint. Coronal FS T2WI (b) shows the lesion effusion in the left hip joint, with mild pericapsular soft
to have a mixed dark intensity to hyperintensity. The tissue edema and enhancement around the anteroinferior
lesion is hyperintense on axial T1WI (c) and heteroge- recess
c d
Fig. 6.2(continued)
a b
Fig. 6.3 Tenosynovial giant cell tumor, localized type. mass (arrows) to exhibit a heterogeneous (intermediate to
Radiograph (a) reveals a nonspecific periarticular soft tis- hyperintense) signal, with typical dark signal foci that
sue mass (arrow) along the ulnar aspect of the proximal correspond to hemosiderin deposition. This mass arises
phalanx of the long finger. Note the mildly expansile between the flexor tendon (star) and bone with adjacent
osteolytic change (arrowheads) at the adjacent phalangeal bone erosion (thin arrows)
head. Axial (b) and sagittal (c) T2WIs show the lobulated
a b
Fig. 6.4 Tenosynovial giant cell tumor, localized type. shows the mass to have marked hypointensity with inho-
Radiograph (a) reveals a nonspecific periarticular soft tis- mogeneous contrast enhancement on postcontrast FS
sue swelling (arrow) around the distal interphalangeal T1WI (c). This lesion is located between bone and flexor
joint with well-demarcated multifocal osteolytic changes tendon with large bone erosion (arrowheads)
(arrowheads) in the neighboring bones. Axial T2WI (b)
a b
Fig. 6.5Tenosynovial giant cell tumor, localized type. some hypointense to dark areas (thin arrow). These regions
Sagittal FS T2WI of the thigh (a) shows an extra-articular correspond to hemosiderin deposition. Axial T1WI (b)
ovoid soft tissue mass (arrows). This mass is located along shows the mass to be isointense relative to skeletal muscle,
the ventral surface of the semimembranosus tendon (arrow- with inhomogeneous enhancement on postcontrast FS
heads). It is hyperintense relative to skeletal muscle, with T1WI (c)
6.4.2 Tenosynovial Giant Cell Tumor, Diffuse Type
a b
c d
Fig. 6.6 Tenosynovial giant cell tumor, diffuse type. FST1WI(c).Axial GRE image (d) demonstrates a
Coronal FS T2WI of the shoulder (a) shows numerous, blooming effect of the low signal nodules (arrows). The
diffuse, variably sized low signal nodules (arrows) within nodules appear larger and darker than they do on
the glenohumeral joint, in addition to synovial T2WI.Radiograph (e) shows periarticular increased
thickening.The lesions have heterogeneous hypointense opacity with neighboring normal bone density.
or slightly hyperintense signal on coronal T1WI (b) with Arthroscopic photograph (f) reveals marked villous,
inhomogeneous contrast enhancement on postcontrast frond-like, thick, yellowish-red synovial proliferation
e f
Fig. 6.6(continued)
a b
Fig. 6.7 Tenosynovial giant cell tumor, diffuse type. observed on T2WI (c), ranging from a dark signal to one
Radiograph (a) shows multilobulated soft tissue mass that is hyperintense to the muscle. Heterogeneous but
around the elbow. Extensive bone erosion is seen, but the intense contrast enhancement is observed on postcontrast
joint spaces are preserved and perilesional bone density is T1WI (d). Sagittal GRE image (e) demonstrates a bloom-
normal. The lesions are isointense on coronal T1WI (b), ing effect of the low signal foci or regions (thin arrows)
with small dark signal foci. A heterogeneous signal is
c d
Fig. 6.7(continued)
6.4.3 Giant Cell Tumor ofSoft Tissue
a b
c d
Fig. 6.8 Giant cell tumor of soft tissue. Axial T2WI of T1WI (d) shows inhomogeneous contrast enhancement of
the thigh (a) shows a circumscribed, extra-articular oval the mass. A fatty rim (arrowheads) around the mass is
soft tissue mass within the right iliopsoas muscle, with a noted. US (e) reveals a slightly heterogeneous hypoechoic
subfascial location. This lesion has mild hyperintensity to mass. A photograph of the specimen (f) reveals a well-
the muscle on sagittal T1WI (b) and heterogeneous hyper- encapsulated, pale yellowish mass with multifocal, irreg-
intensity with dark areas (thin arrow) corresponding to ularly shaped, intense sun-yellow areas. In addition,
hemosiderin deposition on T2WI (a, c). Postcontrast FS multiple hemorrhagic foci are shown on the cut surface
e f
Fig. 6.8(continued)
6.4.4 Deep Benign Fibrous Histiocytoma
a b
c
e
Fig. 6.9Deep benign fibrous histiocytoma. Lateral on T1WI (b) and hyperintense on T2WI (c). Axial (d) and
radiograph (a) reveals a nonspecific soft tissue mass sagittal (e) postcontrast FS T1WIs show the mass to have
(arrow) along the palmar aspect of the distal phalanx. mild peripheral thick enhancement, with a central, large
There is no osseous involvement. This well-circumscribed non-enhancing area. Perilesional soft tissue edema and
subcutaneous mass is isointense to slightly hyperintense enhancement (arrowheads) are also seen
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rare tumor indicating occasional metastatic potential.
Thomas KL, Henderson-Jackson E, Caracciolo JT.Test
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yourself: answerdeep benign fibrous histiocytoma.
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DL.Pigmented villonodular synovitis: MRI character-
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M.Giant cell tumor of the tendon sheath (nodular
Jo E, Cho ES, Kim HS, Nam W.Deep benign fibrous his-
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doi:10.5125/jkaoms.2015.41.5.270.
Smooth Muscle Tumors
7
7.1 eiomyoma ofDeep Soft

L tered calcifications. Sonographic findings include
Tissue a well-defined, noncompressible, heterogeneous,
relatively hypoechoic mass with or without intra-
Leiomyoma of deep soft tissue is a rare benign lesional vascularity on color or power Doppler
neoplasm that occurs in deep soft tissue in the images (Paluck etal. 2015). On MR imaging, this
retroperitoneum or abdominal cavity. This tumor lesion is isointense or slightly hyperintense on
is composed of orderly intersecting fascicles of T1-weighted images and hyperintense or some-
spindled to slightly epithelioid cells, similar to what hypointense on T2-weighted images, with
normal smooth muscle cells. Most lesions are prominent contrast enhancement (Bommireddy
paucicellular, and degenerative or regressive and Gurram 2016; Chalidis and Dimitriou 2007;
changes, including fibrosis, calcification, hyalin- Jalgaonkar etal. 2012; Ramachandran etal.
ization, and myxoid changes, are common in 2014).
large lesions. It is known that calcification, hya-
linization, and myxoid changes are common for
this lesion (Miettinen and Quade 2013). 7.2 Leiomyosarcoma
Leiomyomas of deep soft tissue present as a
large mass and/or pain. Leiomyomas of deep soft Leiomyosarcoma is a rare malignant mesenchy-
tissue of the retroperitoneum or abdominal cavity mal neoplasm showing pure smooth muscle dif-
occur almost exclusively in women, primarily in ferentiation, comprising approximately 10% of
young adulthood or middle age. The very rare all soft tissue sarcomas and being the second or
leiomyomas in the deep peripheral soft tissue third most common soft tissue sarcoma (Salas
occur at almost any age and exhibit no sex predi- etal. 2009; Kransdorf 1995). Soft tissue smooth
lection. Leiomyomas rarely occur in the extremi- muscle tumors with both nuclear atypia and
ties and are more common in the lower limb than mitotic activity are generally diagnosed as leio-
in the upper extremities (Robinson and Kalish myosarcoma, acknowledging the potential for
1990). The treatment of choice is complete surgi- metastasis (Miettinen 2014). Leiomyosarcomas
cal excision of the tumor along with the sur- are subclassified into uterine; soft tissue, which
rounding muscle. The rate of recurrence is very includes cutaneous, major vessel, and deep soft
rare, and this tumor rarely undergoes malignant tissue; and bone leiomyosarcomas (Weiss 2002).
transformation (Miettinen and Quade 2013). Leiomyosarcoma of the soft tissue presents
Imaging findings of leiomyoma of deep soft with a mass lesion. Leiomyosarcomas usually
tissue are nonspecific. Radiograph may demon- occur in the fifth and sixth decade of life (Svarvar
strate soft tissue swelling with intralesional scat- etal. 2007; Massi etal. 2004; Kransdorf 1995).

122 7 Smooth Muscle Tumors
Uterine and retroperitoneal leiomyosarcomas Radiologic findings of leiomyosarcoma are

show a marked female predominance, but no sex usually not specific. Radiographs demonstrate a
predilection at other sites. These tumors most soft tissue mass. Mineralization may be uncom-
commonly occur in the uterus and retroperito- monly observed and is caused by either nonneo-
neum, and less commonly in the viscera, bone, plastic ossification or dystrophic mineralization in
soft tissue, and skin (Weiss 2002). According to the tumor (Bush etal. 2003). Sonography, CT, and
the largest recent series from the Scandinavian MR imaging demonstrate a well-circumscribed
Sarcoma Group (Svarvar etal. 2007), non- soft tissue mass with a variable degree of necrotic
visceral leiomyosarcomas occur most commonly or cystic change, hemorrhage, and contrast
in the extremities, followed by the trunk wall and enhancement (Beaman etal. 2007; Gordon etal.
head/neck. Regarding tumor depth, subcutaneous 2014; Kransdorf 2006). Deep soft tissue leiomyo-
involvement (45%) is more common, followed sarcomas tend to be larger than superficial leio-
by deep-seated (39%) and cutaneous (16%). myosarcoma and heterogeneous in enhancement
Patients with superficial leiomyosarcoma have because of internal hemorrhage and necrosis. Due
longer survival, which is likely related to the to their smaller size at presentation, the superficial
small size and earlier diagnosis of these tumors (i.e., subcutaneous) tumors are homogeneous in
(Gordon etal. 2014; Svarvar etal. 2007). Deep enhancement (Gordon etal. 2014).
soft tissue leiomyosarcoma of the extremities, in
contrast to leiomyosarcomas arising at other
sites, has a greater propensity for local recur- 7.3 I llustrations: Smooth Muscle
rence, with a rate of approximately 27% Tumors
(Lamyman etal. 2011). The metastasis rate is
20% for subcutaneous and 60% for deep intra- 7.3.1 L
eiomyoma ofDeep Soft
muscular leiomyosarcomas (Svarvar etal. 2007). Tissue
a b
Fig. 7.1 Leiomyoma of deep soft tissue. Sagittal T1WI observed on postcontrast FS T1WI (c, d). US shows a
(a) shows a well-demarcated, multilobulated soft tissue smoothly marginated solid mass with mixed hypo- and
mass (star) that is located in the subcutis of the lateral hyperechogenicity and prominent intralesional blood flow
aspect of the right midfoot. This lesion is slightly hypoin- on the color Doppler image (e). A photograph of the spec-
tense to isointense relative to skeletal muscle on T1WI (a) imen (f) shows a circumscribed, multilobulated, yellowish
and heterogeneously isointense to hyperintense on T2WI mass with multifocal peripheral mucoid change (star) and
(b). Inhomogeneous intense contrast enhancement is brown areas (arrowheads)
c d
e f
Fig. 7.1(continued)
Fig. 7.2 Leiomyoma of

deep soft tissue. Axial a b
T1WI (a) shows a
circumscribed
intramuscular soft tissue
mass (arrow) arising
from the lumbrical
muscle of the hand. This
lesion is hyperintense
relative to skeletal
muscle on T1WI with
homogeneous intense
contrast enhancement
(b). Sagittal T2WI (c)
shows a smoothly
marginated oval mass to
have heterogeneous,
mildly hyperintense
signal relative to skeletal
muscle, with small
hypointense foci
c
a b
c d
Fig. 7.3 Leiomyoma of deep soft tissue. Radiograph (a) Strong enhancement is observed on axial postcontrast FS
shows an intra-articular soft tissue mass medial to the T1WI (d). Note the multiple nodular dark-signal calcifi-
medial femoral condyle. The tumor is isointense to mus- cations (arrow) and adjacent bone erosion, with a thin
cle on sagittal T1WI (b) and has mixed signal intensity on sclerotic rim (arrowheads)
T2WI (c), ranging from hypointensity to hyperintensity.
7.3.2 Leiomyosarcoma
a b
c d
e f
vein
Fig. 7.4 Leiomyosarcoma. Axial T1WI (a) shows a to intratumoral vascularities. Axial postcontrast FS T1W
well-demarcated soft tissue mass (star) that is located in (c) shows the mass to have inhomogeneous intense con-
the intermuscular fat plane among the muscles of the trast enhancement. US (e) reveals a circumscribed hyper-
thigh. This mass is intimately associated with the superfi- echoic solid mass (star) with intratumoral anechoic
cial femoral vessels (arrowheads), with craniocaudal tubular structures and internal blood flow on the color
extension along the vessels on axial (b) and sagittal (d) Doppler image (f), in keeping with intratumoral vessels.
T2WIs. It is isointense to slightly hyperintense to skeletal Note the intraluminal tumor extension into the adjacent
muscle on T1WI (a), with inhomogeneous low to interme- superficial femoral vein. A photograph of the specimen
diate hyperintense signal intensity relative to skeletal (g) shows a well-circumscribed mass with a whitish cut
muscle on T2WI (b, d). There are central and peripheral surface and multifocal blood-filled vessels. Direct tumor
tubular signal void structures (thin arrows) corresponding extension into the adjacent vein is shown
Fig. 7.4(continued)
Fig. 7.5Leiomyosarcoma.
Axial T1WI (a) shows a a c
well-demarcated soft tissue
mass located within the
adductor magnus muscle of
the thigh. This lesion is
mildly hyperintense relative
to skeletal muscle, with
focal hyperintense foci
(arrowhead) that correspond
to hemorrhaging. Axial
T2WI (b) reveals the mass
to have heterogeneous
hypo- to hyperintense signal
intensity relative to skeletal
muscle. Small dark foci b
(thin arrows) that suggest
vessels are seen, as is a
large, fluid-equivalent
hyperintense cystic or
necrotic change (star). In
addition to a hypermetabolic
primary mass in right thigh,
PET (c) reveals a
hypermetabolic lung
metastasis (white arrow).
Gross specimen photograph
(d) shows a well-
circumscribed flesh-colored
and yellowish mass with a
large hemorrhagic, necrotic d
or cystic change and
intratumoral vascularities
(thin arrows)
a b
Fig. 7.6 Cutaneous leiomyosarcoma. Postcontrast CT scan (a) shows a small exophytic skin nodule from the posterior
shoulder, with peripheral rim enhancement. PET (b) shows the lesion to be hypermetabolic
a b
Fig. 7.7 Leiomyosarcoma. Sagittal FS T2WI (a) shows a (c) Inhomogeneous enhancement is observed on postcon-
well-demarcated inhomogeneous hyperintense soft tissue trast FS T1WI (d). US (e) shows the mass to be heteroge-
mass (relative to skeletal muscle) closely abutting the neously hypoechoic, with posterior sonic enhancement.
proximal Achilles tendon. The mass has mild hyperinten- The appearance of the mass is nonspecific. However, PET
sity to muscle on axial T1WI (b) and a heterogeneous sig- reveals the mass to be fairly hypermetabolic (SUV 8.0)
nal on T2WI, ranging from intermediate to hyperintense.
References 129
c d
e f
Fig. 7.7(continued)
misdiagnosis and mismanagement. World JSurg

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Pericytic (Perivascular) Tumors
8
Pericytes are contractile cells that wrap around treatment for glomus tumors is complete exci-
the endothelial cells of capillaries and venules sion, and clinical outcomes are generally good.
throughout the body. These supportive perivas- Radiographs detect a small soft tissue mass
cular cells are essential to angiogenesis, tissue located along the dorsal surface of the finger,
homeostasis, vessel permeability, and blood either medially or laterally, related to the nail bed
pressure control (Scott etal. 2015). Pericytic/ and occasionally with extrinsic bone erosion. The
perivascular soft tissue tumors are generally common sonographic findings of subungual glo-
composed of modified vascular smooth muscle mus tumors are solid, hypoechoic or isoechoic
cells and tend to grow in a circumferential fash- nodules beneath the nail plate; hypervascularity
ion around blood vessels (Scott etal. 2015; on color or power Doppler imaging; and occa-
Flope etal. 2013). sionally neighboring bony erosion. MR imaging
reveals small soft tissue nodules with intermedi-
ate or low signal intensity on T1-weighted
8.1 Glomus Tumor images, marked hyperintensity on T2-weighted
images, and strong enhancement after intrave-
Glomus tumors are benign tumors arising from a nous contrast (Baek etal. 2010).
contractile neuromyoarterial glomus body, a spe- Solid glomus tumors comprise approximately
cialized arteriovenous anastomosis involved in 75% of all glomus tumors, consisting of nests of
thermoregulation. These tumors account for glomus cells that surround capillary-sized ves-
approximately 2% of the soft tissue tumors, and sels. One variant, known as glomangioma or glo-
most are diagnosed between the age of 30 and muvenous malformation, is composed of typical
50years. The prevalence of glomus tumors is glomus cells but has a similar structure to a cav-
nearly equal in men and women, except for a ernous hemangioma. Another variant, glomangi-
female predilection in subungual lesions. Most of omyoma, shows partial or focal smooth muscle
these tumors occur in the distal extremities, espe- differentiation. Glomangiomatosis (diffuse glo-
cially the subungual region, hand, wrist, and foot, mus tumor) is extremely rare and defined as (1)
but rare tumors have been reported in almost diffuse growth resembling angiomatosis, with a
every location. Clinically, glomus tumors are prominent glomus component investing vessel
small (less than 1cm), red-bluish subungual nod- walls, and (2) a lack of the criteria for a malig-
ules with a classic clinical triad of pain, pinpoint nant glomus tumor or glomus tumor of uncertain
tenderness with blunt palpation, and cold sensi- malignant potential. The conversion of the glo-
tivity (Flope etal. 2013). The gold standard of the mus tumor to a malignant tumor is reported to be

132 8 Pericytic (Perivascular) Tumors
very rare, less than approximately 1%. The cific. US demonstrates a well-circumscribed
criteria for malignant glomus tumor are as fol- hypoechoic or heterogeneous echoic mass in der-
lows: (1) tumors with a deep location, (2) a size mal and subcutaneous tissue, with hypervascular-
more than 2cm, (3) atypical mitotic figures or ity on Doppler images. MR imaging shows
obvious nuclear atypia, and (4, 5) more mitotic heterogeneous, but predominantly hyperintense,
figures/50 high-power fields (Scott etal. 2015; pattern on T2-weighted images and an isointense
Folpe etal. 2001, 2013). signal on T1-weighted images, with strong con-
trast enhancement (Choi etal. 2014; Harish etal.
2007; Kayes etal. 2002). Intralesional hemor-
8.2 Myopericytoma, rhage may be present due to prominent vascular-
IncludingMyofibroma ity (Harish etal. 2007).
Myopericytomas are tumors characterized by a

hemangiopericytoma-like vascular architectural 8.3 Angioleiomyoma
pattern, with features of perivascular myoid
(myopericytic) differentiation, and show a spec- Angioleiomyoma, also referred to as angiomy-
trum of growth patterns that overlap with myofi- oma or vascular leiomyoma, is a benign smooth
broma. These tumors can be histopathologically muscle tumor that arises from the arterial smooth
categorized as myopericytoma or myofibroma muscle cells or the tunica media of the veins and
depending on the predominant growth pattern, is composed of well-differentiated smooth mus-
namely, the presence of a concentric perivascular cle cells with intervening vascular channels
arrangement of plump, spindle shaped cells in (Hachisuga etal. 1984).
myopericytoma and a zonal/biphasic appearance Angioleiomyoma is a common tumor,
in myofibroma (Dray etal. 2006). accounting for approximately 45% of benign
Myopericytoma presents with a painless, soft tissue tumors. It can occur anywhere in the
slowly growing, superficially located nodule. body but is most often observed in the extremi-
They are most often dermal or subcutaneous soli- ties, particularly the lower leg, followed by the
tary tumors typically found in the lower extremi- head and neck. Tumors in the lower extremities
ties and more commonly in men (Scott etal. occur in females twice as frequently as in males,
2015). Myofibromas may present as painless, whereas those in the upper extremities or head
purple to pink, solitary or multicentric lesions are more frequent in males. These tumors can be
(infantile myofibromatosis). Infantile myofibro- located in the dermis, the subcutaneous fat, or the
matosis occurs primarily at birth and in children superficial fasciae of the extremities. Lesions
less than 2years of age. These tumors primarily occur at all ages but are more common in the
arise in dermal and subcutaneous tissues, and the fourth to sixth decades. They present as a small,
extremities, head and neck region, and trunk are slowly growing firm, and mobile nodule. Pain,
the usual sites. Superficial myopericytomas or and/or tenderness, is manifested in approximately
myofibromas tend to follow a benign course, 60% of patients (Hachisuga etal. 1984). Pain is
with few recurring after complete excision, thought to be caused by the active contraction of
whereas deeper-seated tumors, multinodular smooth muscle, resulting inlocal ischemia.
tumors in childhood, and multiple visceral lesions Treatment usually consists of simple excision.
may pursue a more aggressive course (Weiss and Sonographic findings of angioleiomyoma
Goldblum 2001). include a well-circumscribed subcutaneous or
Radiologic findings of myopericytoma and superficial located mass with a parallel orienta-
myofibroma/myofibromatosis are fairly nonspe- tion to the skin; homogeneous hypoechogenicity;
8.4Illustrations: Pericytic (Perivascular) Tumors 133
straight and linear vessels in the tumor, with and an adjacent tortuous vascular structure (Yoo
convergence to one point on color Doppler; and etal. 2009).
the absence of calcifications (Park etal. 2012;
Zhang etal. 2016). MR imaging demonstrates a
well-demarcated subcutaneous mass of isoin- 8.4 Illustrations: Pericytic
tense signal on T1-weighted images and a het- (Perivascular) Tumors
erogeneous high signal intensity on T2-weighted
images, with homogeneous strong enhancement 8.4.1 Glomus Tumor
a b
Fig. 8.1 Glomus tumor. Radiograph (a) shows extrinsic region, with focal bone erosion (thin arrows) in the dorsal
scalloping (thin arrows) of the distal phalanx and a scle- cortex of the distal phalanx. Prominent blood flow is
rotic rim. Longitudinal US (b) reveals a circumscribed observed on the color Doppler image (c)
homogenous hypoechoic nodule (star) in the subungual
a b
Fig. 8.2 Glomus tumor. Sagittal FS T2WI (a) shows a signal intensity on axial T1WI (b) and homogenous
circumscribed, lobulated hyperintense nodule (arrow) in intense enhancement on postcontrast FS T1WI (c).
the subungual region, with marked bony erosion of neigh- Intraoperative photograph (d) shows a faint blue colored
boring distal phalanx. This lesion exhibits hyperintense spot (thin arrows) beneath the nail
a b
c d
Fig. 8.3 Glomus tumor. Axial T1WI (a) shows a small and sagittal (d) postcontrast FS T1WI.Note the dorsal
isointense soft tissue nodule (star) in the subungual region. bone erosion of the neighboring distal phalanx (arrow-
This circumscribed oval lesion is hyperintense on T2WI heads). The specimen photograph (e) shows an oval gray-
(b) and has homogeneous strong enhancement on axial (c) ish-white nodule with a smooth and glossy cut surface
a d
Fig. 8.4 Glomus tumor. Axial T1WI (a) shows a small (b). Prominent heterogeneous contrast enhancement of
subcutaneous soft tissue mass (arrow) in the plantar sub- the mass is observed on postcontrast FS T1WI (c). A spec-
cutaneous fat layer between the big toe and the second imen photograph (d) reveals a yellowish cut surface of the
toe, separate from the neighboring bones. The mass is circumscribed lobulated tumor and multifocal intratu-
isointense relative to skeletal muscle. This lesion is het- moral hemorrhage
erogeneously hypointense to hyperintense on FS T2WI
8.4.2 Glomangioma
a b
c d
Fig. 8.5 Glomangioma. Oblique radiograph (a) reveals a postcontrast FS T1WI (d). Multifocal dark signal voids
nonspecific soft tissue mass (arrow) along the plantar (thin arrows) are seen, corresponding to mineralization.
aspect of the calcaneus. Highly irregular mineralization US (e) shows a solid heterogeneous hypoechoic mass
(thin arrows) is observed within the mass. This well- with prominent blood flow on color Doppler image.
circumscribed subcutaneous mass is slightly hyperintense Multifocal bright hyperechogenic foci (thin arrows) with
on T1WI (b) and heterogeneous hyperintense on T2WI posterior shadowing reflect mineralization
(c). Heterogeneous intense enhancement is observed on
Fig. 8.5(continued)
8.4.3 Glomangiomatosis
a b
Fig. 8.6 Glomangiomatosis. Axial T1WI (a) shows lesions show inhomogeneous, mild hyperintensity on
variably sized, multifocal nodular lesions (arrowheads) T1WI (a) and inhomogeneous hyperintensity on T2WI
involving the skin and superficial subcutaneous fat (b). Postcontrast FS T1WI (c) shows inhomogeneous
layer of the dorsolateral aspect of the upper arm. The intense contrast enhancement
8.4.4 Myofibroma
a b
c d
Fig. 8.7 Myofibroma. Axial T1WI (a) shows a lobulated mixed signal (ranging from dark to fluid-equivalent),
intramuscular soft tissue mass (star) in the vastus medialis brightly hyperintense, with a peritumoral, mildly hyperin-
muscle, with inhomogeneous, mild hyperintensity. On tense area (arrowhead), in keeping with soft tissue edema.
T2WI (b), the circumscribed mass has a heterogeneous Longitudinal US (e) shows a heterogeneous hypoechoic
hyperintense signal and small fluid-equivalent hyperin- solid soft tissue mass (star) with infiltrating margins (thin
tense foci (thin arrows). Postcontrast FS T1WI (c) reveals arrows) and posterior sonic enhancement (arrowheads).
heterogeneous enhancement, i.e., more intense peripheral Some peripheral blood flow is observed on the power
rim enhancement and less enhancement centrally. Doppler image (f). A photograph of the specimen (g)
Multifocal non-enhancing foci (thin arrows) are fluid- shows a circumscribed lobulated mass with a yellowish-
equivalent hyperintense on T2WI, corresponding to cystic white cut surface and small, empty cystic change
changes. Sagittal FS T2WI (d) shows a heterogeneous
e f
Fig. 8.7(continued)
a b
c d
Fig. 8.8 Myofibroma. Coronal T1WI of the hand (a) geneous hyperintensity on FS T2WI (b). Coronal (c) and
reveals a small, well-described, slightly hyperintense (to axial (d) postcontrast FS T1WIs demonstrate early
muscle) nodule affecting the skin and the hypodermal fat peripheral thick rim enhancement and delayed, gradual
tissue of the digital web space. The lesion shows inhomo- central fill-in enhancement
8.4.5 Myopericytoma
a f
Fig. 8.9 Myopericytoma. Axial T2WIs (a, b, c) of fourth and sagittal (f) postcontrast FS T1WIs demonstrate multi-
finger show multifocal soft tissue lesions (arrows) in the focal inhomogeneous enhancing lesions along the long
palmar subcutaneous tissue. The lesions shows heteroge- axis of the affected finger. Specimen photograph (g)
neous signal, ranging from isointense to hyperintense to shows white or reddish-white colored soft tissue lesions
muscle on T2WI and isointense on T1WI (d). Axial (e) with mildly glossy cut surfaces
8.4.6 Angioleiomyoma
a b
c d
Fig. 8.10 Angioleiomyoma. Sagittal T1WI (a) reveals a (c, d) show strong contrast enhancement. Note that the
circumscribed, lobulated subcutaneous mass (star), which lesion is closely abutting the surrounding subcutaneous
is heterogeneously hyperintense on T2WI (b) and exhibits vascular structure (arrowheads)
tubular signal voids (thin arrows). Postcontrast FS T1WIs
a b
c d
Fig. 8.11 Angioleiomyoma. AP radiograph (a) reveals a taneous signal changes (arrowheads) are noted. US (e)
bulging, contoured soft tissue mass in the medial aspect of reveals marked heterogeneous hyperechogenicity with
the knee. There is no mineralization within the lesion. multifocal anechoic tubular structures, which are observed
Compared to skeletal muscle, the lesion shows a hetero- as blood flow on the color Doppler image (f). Specimen
geneous hyperintense signal on FS T2WI (b, d) and mild photo (g) shows a sharply circumscribed, whitish-yellow
hyperintensity on T1WI (c). Tubular signal voids (thin mass with multiple blood-filled channels (arrowheads)
arrows) are observed within the mass. Perilesional subcu- and hemorrhages
e f
a b c
d e
Fig. 8.12Angioleiomyoma. Axial T1WI (a) of the observed on postcontrast FS T1WI (c). US (d) reveals a
ankle shows a circumscribed, isointense subcutaneous homogenous hypoechoic lesion with compression due to
lesion near the Achilles tendon. The lesion is hyperin- mild pressure. Color Doppler image (e) reveals intratu-
tense on FS T2WI (b), and strong enhancement is moral hypervascularity
References 147
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Flanagan AM.Myopericytoma in Kagers fat pad.
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Choi JH.Subungual tumors: clinicopathologic corre-
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Choi GW, Yang JH, Seo HS, Kim WT, Lee MJ, Yoon
MH.Sonographic appearances of soft tissue angi-
JR.Myopericytoma around the knee: mimicking a
oleiomyomas: differences from other circumscribed
neurogenic tumour. Knee Surg Sports Traumatol
soft tissue hypervascular tumors. JUltrasound Med.
Arthrosc. 2014; doi:10.1007/s00167-014-3390-x.
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Dray MS, McCarthy SW, Palmer AA, Bonar SF,
Scott MA, Shen J, Lam K, Yen Y-H, Mravic M, Lugassy
Stalley PD, Marjoniemi V, Millar E, Scolyer
C, James AW.Review of pericytes in tumor biology.
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broma. A review of 14 cases. JClin Pathol.
Weiss SW, Goldblum JR.Myofibroma and myofibroma-
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tosis. In: Enzinger and Weisss soft tissue tumors. St
Flope AL, Brems H, Legius E.Glomus tumours. In:
Louis, MO: Mosby; 2001. p.35763.
WHO classification of tumours of soft tissue and
Yoo HJ, Choi JA, Chung JH, Oh JH, Lee GK,
Choi JY, Hong SH, Kang HS.Angioleiomyoma in soft
on Cancer; 2013. p.1167.
tissue of extremities: MRI findings. AJR Am
Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss
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SW.Atypical and malignant glomus tumors: analysis
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of 52 cases, with a proposal for the reclassification of
Zhang JZ, Zhou J, Zhang ZC.Subcutaneous angioleio-
glomus tumors. Am JSurg Pathol. 2001;25(1):112.
myoma: clinical and sonographic features with histo-
Hachisuga T, Hashimoto H, Enjoji M.Angioleiomyoma.
pathologic correlation. JUltrasound Med. 2016;35(8):
A clinicopathologic reappraisal of 562 cases. Cancer.
166973. doi:10.7863/ultra.15.06056.
1984;54(1):12630.
Skeletal Muscle Tumors
9
9.1 Rhabdomyosarcoma occurs in adolescents and young adults. It more

commonly affects the extremities (Harms 1995).
Rhabdomyosarcomas are malignant soft tissue Pleomorphic rhabdomyosarcoma is a high-
tumors showing skeletal muscle or rhabdomyo- grade sarcoma composed of sheets of large, atyp-
blastic differentiation. The WHO classifies rhab- ical, bizarre, and frequently multinucleated
domyosarcoma into four types as follows: polygonal eosinophilic cells or of undifferenti-
embryonal, alveolar, pleomorphic, and spindle ated round to spindle cells that display skeletal
cell/sclerosing. muscle differentiation without embryonal or
Embryonal rhabdomyosarcoma has pheno- alveolar components (Montgomery and Barr
typical and biological features of embryonic 2013). This type usually occurs in adults, with a
skeletal muscle cells, being composed of high prevalence of patients in the sixth to seventh
rounded-short spindle cells, often with loose decades of life. Pleomorphic rhabdomyosarcoma
myxoid foci, and containing variable numbers of is most frequently encountered in the deep soft
eosinophilic rhabdomyoblasts. It is the most tissue, most often in the lower extremities (Allen
common type of all rhabdomyosarcomas, pri- etal. 2007; Hawkins etal. 2001).
marily occurring in the first decade of life. Spindle cell/sclerosing rhabdomyosarcoma is an
Embryonal rhabdomyosarcoma is more common uncommon type, being predominantly composed of
in males, with a male-to-female ratio of 1.4:1. It spindled neoplastic cells with infiltrative edges, with
most frequently occurs within the head and neck, a fascicular or storiform growth pattern. Occasionally,
followed by the genitourinary system. In general, this lesion may show focal, subtotal, or total stromal
the embryonal type shows the most favorable hyalinization, with tumor cells arranged in nests,
prognosis, and children have a better outcome microalveoli, or trabeculae, imparting a pseudovas-
than adults (Parham etal. 2013; Ognjanovic etal. cular appearance (Nascimento and Barr 2013). This
2009; Van Rijn etal. 2008). type affects both children and adults, with a strong
Alveolar rhabdomyosarcoma consists of uni- male predilection. Spindle cell/sclerosing rhabdo-
form population of primitive cells with round myosarcoma most frequently involves the parates-
nuclei in an alveolar pattern or in diffuse sheets ticular regions in children, while it usually affects in
with wreath-like multinucleated giant cells the head and neck region in adults. It has a favorable
(Parham and Barr 2013; Allen etal. 2007; outcome in children, whereas it does not in adults
Hawkins etal. 2001). This type is a high-grade due to the high rate of recurrence and metastasis.
sarcoma with a high rate of early regional and There are no specific radiologic imaging find-
distant metastasis. Alveolar rhabdomyosarcoma ings for rhabdomyosarcoma. On CT and MR
is the second most common type and primarily imaging, it usually manifests as a nonspecific soft

150 9 Skeletal Muscle Tumors
tissue mass, with occasional intralesional necro- 9.2 I llustrations: Skeletal Muscle
sis and heterogeneous contrast enhancement. Tumors
Areas of hyperintense signal on T1-weighted
images may show a degree of hemorrhaging, pro- 9.2.1 Embryonal
tein, or hyaline collagenous stroma (Saboo etal. Rhabdomyosarcoma
2012; Van Rijn etal. 2008).
a b
c d
Fig. 9.1Embryonal rhabdomyosarcoma. Coronal FS and some solid mural enhancement on postcontrast FS
T2WI (a) of the pelvis shows a circumscribed mass within T1WI (d). Peritumoral FS T2 shows associated hyperin-
the gluteus maximus muscle. The lesion exhibits mixed tense soft tissue edema (arrowheads). A mildly T1 hyper-
iso- and hyperintensity to muscle on T1WI (b), heteroge- intense region with an intermediate T2 signal and no
neous hyperintensity on T2WI (c), and a peripheral rim enhancement (star) represents a hemorrhage
9.2Illustrations: Skeletal Muscle Tumors 151
9.2.2 Alveolar Rhabdomyosarcoma
a c
e
b
Fig. 9.2 Alveolar rhabdomyosarcoma. Radiograph (a) bones shows slight hyperintensity to muscle on T1WI (c),
shows extrinsic scalloping of adjacent metatarsal bones heterogeneous intermediate to hyperintensity on T2WI
by the soft tissue mass. Color Doppler image (b) reveals a (d), and inhomogeneous enhancement on postcontrast FS
circumscribed, inhomogeneous hypoechoic mass with T1WI (e). It is accompanied by bone marrow signal alter-
intralesional hypervascularity and posterior sonic ation in the neighboring metatarsal bones, without corti-
enhancement. The mass located between the metatarsal cal destruction
9.2.3 Pleomorphic Rhabdomyosarcoma
a b
Fig. 9.3 Pleomorphic rhabdomyosarcoma. Radiograph FS T1WI (d) shows the mass to have internal heteroge-
(a) shows shallow cortical scalloping (arrowheads) of the neous hyperintensity, but a subtracted image (e) reveals
tibial shaft. Axial T1WI (b) reveals a circumscribed mass peripheral rim enhancement. The cut surface of the gross
between the tibia and fibula, with a mixed signal that specimen reveals a large hemorrhagic necrotic area (more
ranges from dark to hyperintense. The lesion has dark sig- than 50%) in the mass (not shown)
nal on T2WI (c) and has hyperintense foci. Postcontrast
a d
Fig. 9.4 Pleomorphic rhabdomyosarcoma. Axial T1WI and psoas muscles. Axial (c) and coronal (e) postcontrast
(a) of the pelvis reveals an inhomogeneously isointense T1WIs reveal heterogeneous and irregular peripheral and
soft tissue mass in the right iliac fossa, with intralesional septal enhancement. PET (f) shows the mass to be strik-
foci that are slightly hyperintense (arrowhead). Axial (b) ingly hypermetabolic (SUV 10.7). A photograph of the
and coronal (d) FS T2WIs show the mass to be heteroge- specimen (g) shows the dark brown-red-yellowish soft tis-
neously hyperintense and involve the neighboring iliacus sue mass with a large intratumoral hemorrhage
f g
Fig. 9.4(continued)
9.2.4 Spindle Cell Rhabdomyosarcoma
a b
c d
Fig. 9.5 Spindle cell rhabdomyosarcoma. Axial T1WI with a partially infiltrative border. Sagittal FS T2WI (d)
(a) of the thigh shows a slightly hyperintense (relative to reveals the multilobulated contour of the lesion, promi-
skeletal muscle) intramuscular mass in the sartorius mus- nent peripheral signal flow voids (thin arrows), and cra-
cle. The mass has heterogeneous hyperintensity on T2WI niocaudally oriented, peritumoral edema (arrowheads)
(b) and strong enhancement on postcontrast FS T1WI (c),
References Hogendoorn PCW, Mertens F, editors. WHO classifi-

cation of tumours of soft tissue and bone. 4th ed.
Lyon: International Agency for Research on Cancer;
Allen SD, Moskovic EC, Fisher C, Thomas JM.Adult
2013. p.1003.
rhabdomyosarcoma: cross-sectional imaging findings
Ognjanovic S, Linabery AM, Charbonneau B, Ross
including histopathologic correlation. AJR Am
JA.Trends in childhood rhabdomyosarcoma inci-
JRoentgenol. 2007;189(2):3717. doi:10.2214/AJR.07.
dence and survival in the United States, 19752005.
2065.
Cancer. 2009;115(18):421826. doi:10.1002/cncr.
Harms D.Alveolar rhabdomyosarcoma: a prognostically
24465.
unfavorable rhabdomyosarcoma type and its neces-
Parham DM, Barr FG.Alveolar rhabdomyosarcoma. In:
sary distinction from embryonal rhabdomyosarcoma.
Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens
Curr Top Pathol. 1995;89:27396.
F, editors. WHO classification of tumours of soft tis-
Hawkins WG, Hoos A, Antonescu CR, Urist MJ, Leung
sue and bone. 4th ed. Lyon: International Agency for
DH, Gold JS, Woodruff JM, Lewis JJ, Brennan
Research on Cancer; 2013. p.1003.
MF.Clinicopathologic analysis of patients with adult
Saboo SS, Krajewski KM, Zukotynski K, Howard S,
rhabdomyosarcoma. Cancer. 2001;91(4):794803.
Jagannathan JP, Hornick JL, Ramaiya N.Imaging fea-
Montgomery EA, Barr FG.Pleomorphic rhabdomyosar-
tures of primary and secondary adult rhabdomyosar-
coma. In: Fletcher CDM, Bridge JA, Hogendoorn
coma. AJR Am JRoentgenol. 2012;199(6):W694703.
PCW, Mertens F, editors. WHO classification of
doi:10.2214/AJR.11.8213.
tumours of soft tissue and bone. 4th ed. Lyon:
Van Rijn RR, Wilde JC, Bras J, Oldenburger F, McHugh
International Agency for Research on Cancer; 2013.
KM, Merks JH.Imaging findings in noncraniofacial
p.1003.
childhood rhabdomyosarcoma. Pediatr Radiol. 2008;
Nascimento AF, Barr FG.Spindle cell/sclerosing rhabdo-
38(6):61734. doi:10.1007/s00247-008-0751-y.
myosarcoma. In: Fletcher CDM, Bridge JA,
Vascular Tumors
10
Pathologists, clinicians, and radiologists have that arises in a synovium-lined surface; the most
traditionally confused the diagnosis of vascular common site is the knee. Intramuscular angioma
anomalies. The International Society for the is referred to as intramuscular hemangioma or
Study of Vascular Anomalies (ISSVA) advocated intramuscular infiltrating angiolipoma and is a
a classification that divided vascular anomalies benign proliferation of blood vessels within skel-
into the following two major categories: tumors etal muscles. This type of hemangioma is primar-
and malformations. This classification system is ily associated with variable amounts of mature
now widely accepted for properly diagnosing and adipose tissue. It is one of the most common
treating vascular anomalies (Sepulveda and deep-seated soft tissue tumors, and occurs most
Buchanan 2014; Lowe etal. 2012). According to frequently in the lower limbs. Venous hemangi-
this classification system, vascular tumors oma is rare and composed of variable-sized veins
(except congenital hemangiomas) are endothelial with thick muscular walls. It presents in the sub-
neoplasms that grow via endothelial hyperplasia, cutaneous or deep-seated soft tissue and is rela-
are not clinically present at birth, have a period of tively common in the limbs. Arteriovenous
rapid growth, and spontaneously involute (except malformation/hemangioma is a benign vascular
noninvoluting congenital hemangiomas). lesion characterized by the presence of arteriove-
Vascular malformations are composed of capil- nous shunting. It predominantly affects the head
laries, veins, lymphatics, and/or arteries with nor- and neck, followed by the limbs. All types of
mal endothelial cell turnover, are present at birth, hemangiomas primarily affect children and young
and grow proportionally with the child. However, adults. Most hemangiomas present as slowly
vascular tumors will be the focus of this chapter, growing lesions, and synovial hemangioma is
based on 2013 WHO classification of soft tissue often associated with swelling and joint effusion/
tumors. hemarthrosis. Intramuscular angioma is occasion-
ally painful after exercise. The symptoms of
AVM/H are associated with the degree of arterio-
10.1 Hemangiomas venous shunting, which can lead to limb hypertro-
phy, venous distension, increased overlying skin
Based on 2013 WHO classification, hemangio- temperature, heart failure, and even consumption
mas are classified into the following four groups: coagulopathy (Kasabach-Merritt syndrome).
synovial hemangioma, intramuscular angioma, Histopathologically, hemangiomas basically con-
venous hemangioma, and arteriovenous malfor- sist of various-sized vessels, such as the capillary
mation/hemangioma (AVM/H). Synovial heman- (small), cavernous (large), and mixed vessels
gioma is a benign proliferation of blood vessels (Kransdorf and Murphey 2014; Calonge 2013).

158 10 Vascular Tumors
Radiographs are occasionally normal or dem- images and hypointensity on T2-weighted images.
onstrate a nonspecific soft tissue mass. Hemangioma shows diffuse contrast enhance-
Calcifications, such as phleboliths, metaplastic ment of varying degree, from strong solid
ossification, or localized cortical/periosteal hyper- enhancement to gradual centripetal enhance-
trophy, can be noted. A Swiss cheese appearance, ment. Synovial hemangioma is characterized by
with multiple rings and arc-like ossifications with an intra-articular location, often with a juxta-
a coarse trabecular pattern, may be observed in articular location. This lesion is frequently
ossifying hemangiomas. On CT, hemangioma is associated with hemorrhagic synovitis character-
observed as a soft tissue mass, occasionally with ized by effusion or hemarthrosis and synovial
strong enhancing serpentine vascular compo- hypertrophy observed with occasional hemosid-
nents, fat overgrowth, and phleboliths. US may erin deposits on MRI. Intramuscular angioma is
reveal a well-defined soft tissue mass with vari- diagnosed when the hemangioma is located in the
able echogenicity. Hemangiomas may be muscle. Venous hemangiomas show slow-flow
hypoechoic or hyperechoic relative to surround- serpentine vessels and a tendency to be oriented
ing tissue and may have a homogeneous or com- along the long axis of the extremities and
plex appearance. If phleboliths are associated, neurovascular bundle. These hemangiomas also
they appear as hyperechoic spots with posterior have multifocal involvement and are character-
shadowing. Color Doppler imaging also reveals ized by muscle atrophy, with increased
various flow signal patterns. Hemangiomas with subcutaneous fat. AVM/H tends to have clusters
slow blood flow may show only sparse monopha- of serpentine signal voidsonMR imaging.
sic (venous) blood flow or no blood flow signal, Thelack of enhancing soft tissue masses
but a dynamic maneuver, such as a repeated com- distinguishes AVM/H from other hypervascular
pression-decompression technique, can help visu- soft tissue sarcomas (Lowe etal. 2012; Murphey
alize internal blood flow. That is, one can watch etal. 1995).
for collapse or non-visualization of the flow sig-
nal on compression and reappearance of the sig-
nal with gradual decompression. Lesions with 10.2 Angiomatosis
high blood flow reveal high blood flow signals or
networks on color Doppler imaging and high sys- When arteriovenous malformation/hemangiomas
tolic arterial flow pattern on spectral Doppler involve a large segment of the body in a contigu-
imaging. In addition, adjacent dilated draining ous fashion, either (1) by longitudinal extension
veins are frequently noted. MR imaging shows a to affect multiple different tissue planes or (2) by
low-to-intermediate signal intensity, occasionally crossing muscle compartments to affect similar
with areas of hyperintensity, representing slow tissue types, these lesions are termed angioma-
blood flow, hemorrhage, or thrombosis on tosis. This lesion occurs commonly in the first
T1-weighted images. A homogenous or heteroge- two decades of life and in the lower extremities.
neous, intermediate to very high signal intensity is It presents as diffuse, persistent swelling of the
observed on T2-weighted images. Frequently, affected location, which may wax and wane in
intratumoral fat deposition is observed within and size and is affected by strenuous activity. If the
around the lesion. The vascular channels and prominent arteriovenous shunting is associated,
spaces of hemangioma have round/oval, linear, or increased skin temperature, thrill, pulsation, or
serpentine appearance, with either high or low hypertrophy of the affected part can be detected.
signal intensity. Hemorrhagic areas have high sig- The imaging appearance of angiomatosis is simi-
nal intensity on T1- and T2-weighted images or lar to those for previously described hemangio-
fluid-fluid levels. Phleboliths are observed as cir- mas. However, angiomatoses are infiltrative and
cular dark or hypointense nodules. Occasionally, affect multiple tissue planes. In addition,
an intratumoral thrombus can be observed as abundant adipose tissue is frequently involved
areas of mild hyperintensity on T1-weighted (Aviv etal. 2001; Rao and Weiss 1992).
10.6 Kaposi Sarcoma 159
10.3 Lymphangioma response. Surgical resection is the most effective

treatment. Mortality from this tumor is approxi-
Lymphangiomas consist of dilated lymphatic mately 10% due to extensive local disease or
channels and exist in two forms as follows: thrombocytopenia (Lyons etal. 2004).
microcystic (capillary, smaller than 2mm) and On MR imaging, KHE has similar features to
macrocystic (cavernous, larger cysts of variable hemangioma but is characterized by an ill-defined
sizes), commonly referred to as cystic hygroma margin, sunburst-shaped stranding, and no fat
and lymphatic malformation. This lesion pres- deposition. Prominent flow voids representing
ents in the first 2 years of life as a palpable soft vascular channels in the mass are usually
fluctuant soft tissue mass and commonly affects observed (Tamai etal. 2010).
the head and neck or axilla (Dubois and Garel
1999).
Radiographs of lymphangioma reveal a non- 10.5 Retiform
specific soft tissue mass with rare calcifications. Hemangioendothelioma
At US, this lesion appears as a unilocular or mul-
tilocular anechoic or hypoechoic mass, with Retiform hemangioendothelioma (RH) is a
septa of variable thicknesses. In addition, if it is locally aggressive, rarely metastasizing vascular
bloody, chylous, or infected, the lesion may show tumor characterized by distinctive arborizing
internal echogenicity of variable amounts and blood vessels lined by typical hobnail endothelial
degree (Sheth etal. 1987). CT demonstrates a cells. RH is uncommon and primarily found in
unilocular or multilocular mass of low attenua- young to middle-aged adults, with no sex predi-
tion similar to water, with peripheral and septal lection. They present as red/bluish slow-growing
enhancement. On MR imaging, lymphangiomas plaques or nodular tumors that affect the skin and
are observed as lobulated, septated masses with subcutaneous tissue of the distal extremities,
T1-weighted iso- to hypointensity and especially the lower limb. Local recurrences are
T2-weighted bright hyperintensity relative to common in more than 50% of patients because of
skeletal muscle. Peripheral and septal contrast the infiltrative growth pattern of RH, but distant
enhancement is also observed. Internal fluid-fluid metastases are extremely rare. Treatment consists
levels or other debris may be shown due to intra- of surgical excision with adequate surgical tumor
lesional bleeding or inflammation/infection margins and monitoring for local reoccurrence
(Moukaddam etal. 2009). (Zheng etal. 2014; Calonge 2013). The imaging
appearance of RH has not been well documented.
It may show a soft tissue mass that affects the
10.4 Kaposiform skin and subcutaneous tissue, with nodular skin
Hemangioendothelioma thickening on US, CT, or MR imaging.
Kaposiform hemangioendothelioma (KHE) is a

locally aggressive vascular tumor frequently 10.6 Kaposi Sarcoma
associated with Kasabach-Merritt phenomenon
(severe thrombocytopenia). This lesion occurs Kaposi sarcoma (KS) is a locally aggressive,
commonly in the first decade of life with male endothelial tumor or a tumor-like lesion that
predominance and most often involves the manifests as one of four forms as follows: (1)
extremities. It presents with a blue-red mass classic indolent KS, which occurs primarily in
located superficially or in the deep soft tissue. elderly men of Mediterranean/East European and
KHE shows no tendency to regress spontane- Ashkenazi Jews, with male predominance; (2)
ously. Histologically, the tumor consists of irreg- endemic African KS, which affects middle-aged
ular vascular lobules growing in an infiltrative men in equatorial Africa who are not infected
fashion and evokes a dense hyaline stromal with HIV; (3) iatrogenic (organ transplant-related)
KS, which is related to chronic drug- induced ities. Radical surgical excision is the mainstay of
immunosuppression for various diseases; and (4) treatment. Most EHEs are indolent, but 2030%
AIDS-associated KS, the most aggressive form of tumors metastasize (Deyrup etal. 2008).
of the disease, frequently found in homosexual The imaging appearance of EHE is nonspe-
male AIDS patients (Tappero etal. 1993). KS cific. On ultrasonography, EHE appears as
usually presents with purplish, reddish blue or hypoechoic or hyperechoic, with prominent cys-
dark brown multifocal cutaneous patches, tic areas due to hemorrhaging. Doppler imaging
plaques, or nodules occurring frequently in distal may reveal AV shunting. On MR imaging, EHE
extremities. However, KS may also involve dif- may demonstrate imaging features of nonspecific
ferent mucosal sites, lymph nodes, and visceral hypervascular tumors, with frequent flow voids
organs. Musculoskeletal involvement by KS is and no fat deposition (Nuthakki etal. 2007).
infrequent and occurs secondary to local exten-
sion from the cutaneous lesions. Thus, KS of the
soft tissue is almost always accompanied by con- 10.8 Angiosarcoma ofSoft Tissue
comitant noticeable cutaneous changes. Chronic
lymphedema has been reported to promote KS Angiosarcoma is an aggressive vascular malig-
development due to a combination of collateral nancy of endothelial cell origin. It is rapidly pro-
vessels, lymphangiogenesis, and immune impair- liferating, with extensively infiltrating anaplastic
ment (Lee etal. 2014). cells derived from blood vessels, and lines irregu-
The imaging appearance of primary KS of the lar blood-filled spaces. It can be caused by thera-
soft tissue has only been minimally documented. peutic radiation or chronic lymphedema
US reveals solid, vascular, heterogeneous skin or (Sepulveda and Buchanan 2014). AS of soft tis-
subcutaneous soft tissue lesions. In our experi- sue has a peak incidence in the seventh decade of
ence, KS appears as solitary or multifocal plaques life and a male predominance. It most commonly
or nodules that affect the skin and areas below the involves the skin, followed by soft tissues, such
subcutis on MR imaging. They are isointense to as deep muscle of the lower extremities. The
muscle on T1-weighted images and hyperintense lesion manifests with a painful rapidly enlarging
on T2-weighted images with solid vascular con- mass. More than half of the patients with AS die
trast enhancement and spiculated margins within the first year. Local recurrence is observed
(Pantanowitz etal. 2008). KS may be accompa- in 20% of cases and distant metastases in approx-
nied by lymphedema, appearing as a diffuse, imately 50%. These metastases are frequently to
honeycomb, or reticular pattern of subcutaneous the lungs, followed by the lymph nodes, soft tis-
tissue on MR imaging. sues, and bone (Meis-Kindblom and Kindblom
1998).
The imaging characteristics of AS of soft tissue
10.7 Epithelioid overlap with those of other hypervascular soft tissue
Hemangioendothelioma sarcomas. On MR imaging, the tumor may show
more aggressive features of soft tissue masses that
Epithelioid hemangioendothelioma (EHE) is a involve the skin, subcutaneous tissue, or muscle.
rare malignant angiocentric vascular tumor com- Other features include infiltration into the surround-
posed of cords of epithelioid endothelial cells in ing tissue, no fat deposition, intense contrast
a distinctive myxohyaline stroma and arise from enhancement with prominent serpentine flow voids,
medium to large blood vessels. This lesion com- and hemorrhagic areas of T1-hyperintense foci or
monly occurs in the fourth and fifth decades, with fluid-fluid levels. Secondary cystic degeneration
a slight female predominance. EHE manifests as and necrosis are often observed (Moukaddam etal.
a slightly painful mass, frequently on the extrem- 2009). AS of soft tissue may be frequently accom-
panied by lymphedema, appearing as a diffuse, 10.9 Illustrations: Vascular

honeycomb, or reticular pattern of subcutaneous tis- Tumors
sue on MR imaging.
10.9.1 Hemangioma
a b c
Fig. 10.1 Hemangioma. Radiograph of finger (a) shows oval dark signal foci (arrowheads) within the bright
several calcifications with the classic appearance of hyperintense, multiloculated subcutaneous mass with
phleboliths and dystrophic mineralization in an organiz- fluid-fluid levels (small arrows)
ing thrombus. Sagittal FS T2WIs (b, c) show round or
10.9.2 Synovial Hemangioma
a b
c d
Fig. 10.2 Synovial hemangioma. MR image of the knee Multifocal, oval, mildly T1 hyperintense and dark T2 foci
shows a multilobulated, multiloculated intra-articular soft (arrowheads) are noted within some locules. These find-
tissue mass (arrows) with inhomogeneous hyperintensity ings are suggestive of thrombi because there were no
relative to muscle on T1WI (a) and heterogeneous hyper- phleboliths on the radiograph (not shown). Coronal post-
intensity on FS T2WI (b, c). The lesion is located along contrast FS T1WI (d) shows the mass to have heteroge-
the synovial surface of the medial suprapatellar bursa. neous enhancement in the subsynovial space
a b
c d
Fig. 10.3 Synovial hemangioma. Radiograph (a) shows heterogeneously hyperintense on T2WI (d), with intense
irregular ossifications (arrows) near the subgastrocnemius contrast enhancement (e). The lesion affects intra- and
recess of the posterior supracondylar area of the knee. juxta-articular spaces. Hypointense lines within the tumor
Sagittal FS T2WI (b) reveals an ill-defined, multilobular, reflect mineralization on all sequences. Specimen photo
multiseptated soft tissue mass (arrows) with mixed signal (f) shows the irregularly bordered soft tissue mass con-
intensity and mild pressure bone erosion (arrowheads). taining multifocal mineralization, such as calcifications
The lesion is isointense to muscle on axial T1WI (c) and and some ossifications (arrowheads)
e f
10.9.3 Intramuscular Angioma
a b
Fig. 10.4 Intramuscular angioma. Radiograph (a) shows muscle on T1WI (c) and inhomogeneously hyperintense
localized cortical hypertrophy (arrow) of the right distal on T2WI (d), with mild inhomogeneous contrast enhance-
femur, with subtle mineralization (arrowheads). Coronal ment on postcontrast FS T1WI (e). Peripheral fat over-
FS T2WI (b) reveals an ill-defined hyperintense soft tis- growth (arrowheads) is noted. Specimen photo (f) reveals
sue mass (arrows) within the vastus lateralis muscle. Note the intramuscular, irregularly marginated hemorrhagic
the surrounding engorged veins (arrowheads) and small soft tissue mass with multifocal mineralization, including
signal void spots, representing intratumoral mineraliza- ossification (thin arrows)
tion (thin arrow). The tumor is mildly hyperintense to
c d
e f
a b
c d
Fig. 10.5 Intramuscular angioma. Axial (a) and coronal enhancement on postcontrast FS T1WI (d). US (e) reveals
(b) T1WIs show an isointense or slightly hyperintense a central isoechoic mass relative to the muscle echo, with
mass (arrow) within the vastus intermedius muscle, with a focal ill-defined margin and surrounding hyperechoic
surrounding fat overgrowth (arrowheads). The lesion has fat tissue (arrowheads). Color Doppler imaging shows
inhomogeneous hyperintensity on T2WI (c) and intense intralesional focal blood
a b
c d
Fig. 10.6 Intramuscular angioma, cavernous type. MR Direct puncture angiography (d) reveals prolonged con-
images reveal a circumscribed, multilobulated, multilocu- trast stagnation in variably sized blood-filled spaces, with
lated soft tissue mass (arrows) in the vastus lateralis mus- the exception of opacification of a small, early draining
cle. The mass exhibits inhomogeneous iso- and mildly vein (arrowheads). On US (e), the lesion has a multilocu-
hyperintensity on T1WI (a), heterogeneous hyperinten- lated appearance with a mixed appearance, including a
sity on T2WI (b), and irregular peripheral and some solid central hyperechoic thrombosis or clots (star) and
enhancement on postcontrast FS T1WI (c). Variably sized anechoic/hypoechoic fluid
T2 hypointense foci (thin arrows) represent thrombi.
10.9.4 Ossifying Intramuscular Angioma
a b
Fig. 10.7 Ossifying intramuscular angioma. Radiograph inhomogeneous mild contrast enhancement. Intratumoral
(a) of the distal thigh shows an ossified mass with Swiss hyperintense foci (stars) on T1- and T2-weighted images
cheese appearance. Axial T1WI (b) reveals a heteroge- with fat suppression reflect intratumoral fat or fatty marrow
neously hyperintense soft tissue mass (arrow) relative to (ossification). Prominent tubular flow voids (thin arrows)
muscle in the adductor magnus muscle. Prominent cranio- are observed in the mass. A photograph of the specimen (e)
caudal perilesional fat overgrowth (arrowheads) is noted on shows a reddish-yellow mass with large internal ossification
coronal T2WI (c). Axial postcontrast FS T1WI (d) reveals (arrow), intervening hemorrhage and vascular channels
10.9.5 Venous Hemangioma
a b
c d
Fig. 10.8 Venous hemangioma. Sagittal FS T2WI of the reveals reddish-purple plaque in the skin overlying the
thigh (a) shows variably sized vascular channels that pri- mass. A photograph of the specimen (f) shows dilated
marily affect subcutaneous tissue, with beaded and a vascular channels with congestion within a background
somewhat reticular appearance. This lesion is isointense reactive fat overgrowth. According to the surgical record,
to muscle on T1WI (b), hyperintense on T2WI (c), and a large amount of blood gushed out immediately after the
has a faint peripheral rim or reticular enhancement on skin incision
postcontrast FS T1WI (d). Intraoperative photograph (e)
e f
10.9.6 Arteriovenous Malformation/Hemangioma
a b
c d e
Fig. 10.9Arteriovenous malformation/hemangioma. growth. This lesion has mixed signals ranging from flow
Clinical photograph (a) of the medial thigh shows a local- voids to bright hyperintensity on T2WI (c). Variable
ized soft tissue bulging (arrow), with visualization of blu- enhancement is observed on postcontrast FS T1WI (d). In
ish engorged vascular structures (arrowhead). Coronal the specimen photograph (e), variable numbers of small
T1WI (b) reveals subcutaneous tortuous, dilated vascular and large blood vessels are seen, many of which are
channels, with some flow voids and surrounding fat over- dilated with hypertrophied fat tissue
10.9.7 Angiomatosis
a b
Fig. 10.10 Angiomatosis. Teleradiograph of the lower extremity (a) shows extensive soft tissue enlargement. Coronal
T1WI (b) and axial T2WI (c) show extensive multi-tissue plane involvement of hemangiomas of various types
10.9.8 Lymphangioma
a b
c d
Fig. 10.11 Lymphangioma. Coronal FS T2WI of the enhancement on postcontrast FS T1WI (c), with pre-
thigh (a) shows an elongated, branched, multilobulated, served intervening normal vessels. Photograph of the
bright hyperintense, subcutaneous soft tissue mass in the specimen (d) shows that the tumor has a multicystic or
right inguinal region. This lesion (arrows) has isointensity sponge- or bubble-like appearance (arrowheads)
relative to muscle on T1WI (b) and little peripheral rim
a d
f g
Fig. 10.12Lymphangioma, complicated. On MR image heads), representing hemorrhage, and surrounding subcu-
of the shoulder, a large multilobulated, multiseptated cystic taneous reticular edematous change. Postcontrast FS T1WI
mass is observed affecting the subcutaneous and deep axil- (e) shows thick peripheral wall enhancement of the mass
lary tissue. This lesion shows bright hyperintensity on and infiltrative subcutaneous and perifascial enhancement.
T2WI (a), hyperintensity relative to muscle on T1WI (b), On US (f), the mass initially has anechoic, multiloculated
and mild thin peripheral and septal contrast enhancement appearance, with relatively thin and sparse septa. However,
on the subtracted image (c). Two years later, the patient follow-up US (g) shows thickening of the wall and septa of
complained of sudden pain, fever, and increased mass size. the lymphangioma, with abnormal intralocular debris and
Follow-up T2WI (d) reveals further increase in the size of surrounding subcutaneous edematous change. Hemorrhagic
the pre-existing cystic mass, with a fluid-fluid level (arrow- pus was confirmed by US-guided aspiration
10.9.9 Kaposiform Hemangioendothelioma
a b
c d e
Fig. 10.13Kaposiform hemangioendothelioma. Mild peritumoral hyperintense curvilinear structures (small

Radiograph (a) shows diffuse soft tissue swelling of the right arrows) without fat suppression reflect slow-flow vascular
lower leg. Note the localized cortical hypertrophy (arrow) on structures in the adjacent deep intermuscular fat plane and
the medial aspect of the proximal fibula. Coronal T2WI (b) soleus muscle. On US (f), the lesion shows heterogeneous
reveals a central multilobulated, hyperintense soft tissue hyperechogenicity (star) with posterior sonic enhancement
mass (star) with tubular signal voids within the tibialis poste- (arrowheads) compared with hypoechogenicity of the
rior muscle, which is craniocaudally swollen and exhibits affected muscle. Prominent intra- and peritumoral blood
tortuous, dilated flow voids. The tumor is isointense to mus- flows are observed on color Doppler image (g). A photo-
cle on T1WI (c), bright hyperintense on T2WI (d), and graph of the specimen (h) shows irregularly shaped central
strongly enhancing on postcontrast FS T1WI (e). The fibrosis (star) with surrounding areas of multifocal brown-
affected muscle (arrowheads) has an intermediate signal on ish-gray hemorrhaging
T2WI, with less prominent enhancement than the tumor.
f g
10.9.10 Retiform Hemangioendothelioma
a b
c d
Fig. 10.14 Retiform hemangioendothelioma. MR image (small arrows), with mild cortical pressure saucerization
of the ankle shows an irregularly bordered subcutaneous (arrowheads). The tumor also encompasses, but does not
soft tissue mass that closely abuts the distal tibia. The compromise, the subcutaneous superficial vascular struc-
lesion shows isointensity on T1WI (a), heterogeneous ture (a, c). The mass shows heterogeneous signals, rang-
hyperintensity on FS T2WI (b), and intense contrast ing from intermediate to bright hyperintensity on coronal
enhancement on postcontrast FS T1WI (c). The mass T2WI (d).Multifocal intratumoral thin flow void struc-
directly extends beneath the adjacent tibial periosteum tures are noted on all sequences
10.9.11 Kaposi Sarcoma
a b
c d e
Fig. 10.15 Kaposi sarcoma. Clinical photograph (a) of sue and is isointense to muscle on T1WI. (c) A heteroge-
the medial foot shows a dark bluish-purple multinodular neous signal with central irregular hypointensity and
mass. Radiograph (b) demonstrates exophytic, multinod- peripheral hyperintensity is observed on T2WI (d), and
ular skin and subcutaneous mass, without neighboring inhomogeneous avid enhancement is observed on post-
bone erosion or intratumoral mineralization. The lesion contrast FS T1WI (e). Surrounding skin thickening with
primarily affects the skin with secondary hypodermal tis- signal alteration (arrowheads) is noted
10.9.12 Epithelioid Hemangioendothelioma
a b
c d
Fig. 10.16Epithelioid hemangioendothelioma. MR observed on sagittal T2WI (d). Irregularly shaped hypoin-
image of the elbow reveals a circumscribed intermuscular tense areas (arrowheads) are histologically confirmed as
soft tissue mass in the subfascial location of the distal ossifications. US (e) shows the well-marginated, mixed
upper arm. The lesion shows mild hyperintensity to mus- echogenic mass with a snowstorm appearance, ranging
cle on T1WI (a), hyperintensity on FS T2WI (b), and het- from hypoechoic areas to hyperechoic foci with posterior
erogeneous contrast enhancement with large shadowing, suggesting intratumoral mineralization.
non-enhancing area on postcontrast FS T1WI (c), sug- Photograph of the specimen (f) shows it as a whitish-
gesting hemorrhage or necrosis. The mass encases, but yellow mass with considerable ossification (arrowheads)
does not compromise, the segmental brachial neurovascu- and multifocal hemorrhagic areas
lar bundles (small arrows). Tubular signal voids are
e f
a b
Fig. 10.17Epithelioid hemangioendothelioma. Axial variably sized, anechoic cystic spaces that are fully filled
T2WI (a) of the thigh shows an irregularly bordered soft with flow signals on the color Doppler image (f), suggest-
tissue mass confined to the rectus femoris muscle, which ing vascular channels. PET (g) shows the mass (arrow) to
is markedly enlarged. The mass is slightly hyperintense to be mildly hypermetabolic with SUV 2.2. The arteriogram
muscle on T1WI (b) and hyperintense on T2WI (c), with (h) reveals marked vascularity with enlarged feeding
avid contrast enhancement on postcontrast T1WI (d). arteries, staining, and early draining veins. Photograph of
Prominent intratumoral flow voids are seen. Note the gross specimen (i) shows a reddish-white mass without
large serpentine, dilated signal void of vascular structures infiltration outside the investing fascia, with variably
outside the tumor, representing AV shunting. US (e) sized vessels and some myxoid change
reveals heterogeneous hypoechoic mass with numerous,
c d
e f
g h i
10.9.13 Angiosarcoma ofSoft Tissue
a b
c d
Fig. 10.18 Angiosarcoma of soft tissue. Radiograph (a) Note the curvilinear dark signal rim (arrowheads) along
of the lower leg shows small curvilinear soft tissue miner- the tumor margin on all sequences, suggesting a hemosid-
alization (arrow). The tumor is observed as an irregularly erin rim. In addition, uneven fat overgrowth (small
bordered, multilobulated mass in the soleus muscle. The arrows) is depicted around the mass (b). On US (e), the
mass exhibits iso- to mild hyperintensity to muscle on mass has heterogeneous hypoechogenicity and an irregu-
T1WI (b), hyperintensity on T2WI (c), and inhomoge- lar and speculated margin. Surrounding hyperechoic fat
neous, but intense contrast enhancement on postcontrast overgrowth (small arrows) is also noted. Color Doppler
FS T1WI (d). Multifocal thin tubular flow voids are seen. image (f) shows intratumoral hypervascularity
e f
Fig. 10.19
Angiosarcoma of soft a d
tissue. MR images (a, b,
c) and radiograph (d)
show diffuse
enlargement of the lower
leg with subcutaneous
edema due to chronic
lymphedema. This
condition developed
after cervical cancer
surgery and radiation
therapy 9years ago.
Multifocal lobular
subcutaneous masses
(arrows) are noted, the b
largest of which shows
exophytic growth. The
mass is heterogeneous
iso- to mildly
hyperintense to muscle
on T1WI (a) and
inhomogeneous,
intermediate to
hyperintense on T2WI
(b) with heterogeneous
contrast enhancement on
postcontrast FS T1WI
(c). A thin dark-signal
rim around the lesions
c
represents hemosiderin
deposition, which can be
observed on all
sequences. T1- and
T2-hyperintense areas
(arrowheads) within the
lesions, not suppressed
on FS sequences,
correspond to
hemorrhagic changes
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Murphey MD, Fairbairn KJ, Parman LM, Baxter KG,
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Chondro-Osseous Tumors
11
Chondro-osseous tumors comprise various types imaging shows a soft tissue mass with hypo- to
of soft tissue lesions and may contain bone or isointensity relative to muscle on T1-weighted
cartilage matrix. Imaging correlation is essential images and hyperintensity on T2-weighted
for soft tissue tumors containing bone or carti- images, representing the high water content of
lage. First and foremost, imaging confirms that the mucopolysaccharide component or myxoid
the lesion originates from the soft tissue. This change (Hondar Wu etal. 2006; Kransdorf and
chapter focuses on chondro-osseous tumors, Meis 1993). Areas with decreased signal inten-
which are defined by the presence of a skeletal sity on T1- and T2-weighted images correspond
matrix in the 2013 WHO classification of soft tis- to calcifications. Postcontrast T1-weighted
sue tumors. images reveal various patterns of enhancement,
ranging from non-enhancement, peripheral and
septal enhancement, to homogeneous enhance-
11.1 Soft Tissue Chondroma ment (Vaseenon etal. 2014; Suganuma etal.
2011; Adaletli etal. 2011; Le Corroller etal.
Soft tissue chondroma (STC) is a benign neo- 2008; Hondar Wu etal. 2006).
plasm consisting of chondrocytes in a hyaline
cartilage matrix, arising in extraosseous and
extrasynovial tissues. Synonyms include 11.2 Extraskeletal Osteosarcoma
extraskeletal chondroma and chondroma of soft
parts. These tumors usually occur in middle age Extraskeletal osteosarcomas or soft tissue osteo-
with a slight male predominance. They affect sarcomas are malignant neoplasms composed of
most frequently the digits, particularly the fin- malignant cells that produce osteoid or bone and
gers, followed by the hands and feet. STCs pres- are located in the soft tissue, without attachment
ent as slowly growing soft tissue masses, with to bone or periosteum. This tumor usually pres-
occasional pain or tenderness. The proper treat- ents later (mid- to late adulthood) compared with
ment is surgical excision, and the prognosis is conventional intraosseous osteosarcoma and has
excellent (Cho and Horvai 2015; Kransdorf and a slight male predominance. The deep soft tissue
Meis 1993). of the lower limbs is most commonly involved,
Radiography shows a well-demarcated soft usually the thigh and buttock regions. The mass
tissue lesion with occasional erosion or periosteal presents as an enlarging soft tissue mass. Most
reaction of the neighboring bone. Focal chon- cases develop de novo, but 413% are associated
droid calcifications with ringlike, stippled, punc- with previous exposure to radiation (Lee etal.
tate, or curvilinear appearances can be seen. MR 1995; Kransdorf and Meis 1993; Chung and

186 11 Chondro-Osseous Tumors
Enzinger 1987). Local recurrence and distant (Cho and Horvai 2015; Varma etal. 1993;
metastasis are common and usually occur by Kransdorf and Meis 1993). The hypointense, non-
3years after excision (Lee etal. 1995). enhancing osteoid component is usually observed
Radiography reveals a soft tissue mass with centrally within the mass or is arbitrarily located.
variable amounts of intratumoral osteoid matrix The central location of the osteoid in the tumor is
mineralization, which appears as an ill-defined, referred to as a reverse zoning phenomenon, in
fluffy, cloud-like, or amorphous region of contradistinction to the peripheral deposition
increased opacity. MR imaging findings are non- (zonal phenomenon) observed in cases of myo-
specific. MR imaging shows a well-demarcated sitis ossificans (Lidang Jensen etal. 1998).
large (515cm) soft tissue mass with mixed
hypointensity on T1-weighted images and mixed,
but predominant hyperintensity, on T2-weighted 11.3 Illustrations: Chondro-
images. Cystic, hemorrhagic non-enhancing, or Osseous Tumors
solid enhancing components can occasionally be
observed on postcontrast T1-weighted images 11.3.1 Soft Tissue Chondroma
a b
Fig. 11.1 Soft tissue chondroma. Lateral radiograph (a) intensity to muscle on T1WI (c), heterogeneous hyperin-
shows soft tissue lesion in the dorsum of the distal pha- tensity on T2WI (d), and inhomogeneous enhancement on
lanx of the thumb, with shallow bone erosion (arrow- postcontrast FS T1WI (e). US (f) shows a hypoechoic
head). There is no mineralization. Sagittal FS T2WI (b) mass and shallow neighboring bone erosion (arrow-
reveals a circumscribed hyperintense soft tissue mass heads). Some intralesional vascularity is depicted on
closely abutting the phalanx, with subtle bone marrow color Doppler image (not shown)
hyperintensity (small arrow). The tumor has slight hyper-
c d e
a b
c d
Fig. 11.2 Soft tissue chondroma. Lateral radiograph of and remodeling of the neighboring bone are noted. The
the finger (a) shows soft tissue lesion (star) in the palmar tumor has mixed signal, ranging from dark to hyperin-
aspect of the proximal phalanx of the middle finger, with tense on T2WI (c), with some peripheral and tiny central
scalloping (arrowheads), periosteal reaction, and bone nodular enhancement on postcontrast FS T1WI (d). The
remodeling (small arrows). No intratumoral calcification specimen photograph (e) reveals a circumscribed, whitish
is seen. Axial T1WI (b) reveals a well-demarcated isoin- firm mass
tense mass between the bone and flexor tendon. Scalloping
a b
c d e
f g
Fig. 11.3 Soft tissue chondroma. Lateral radiograph of tensity. The lesion is isointense to muscle on T1WI (c)
the foot (a) shows a soft tissue lesion in the plantar aspect with irregular thick rim enhancement along the periphery
of the midfoot, with bulging soft tissue contours. on postcontrast FS T1WIs (e, f). Multifocal hypointense
Multifocal rings and arcs as well as curvilinear and foci (arrowheads) on all sequences correspond to calcifi-
popcorn- like calcifications are noted. Sagittal (b) and cation. A photograph of the specimen (g) shows multi-
axial (d) FS T2WIs reveal a circumscribed subcutaneous lobulated yellowish-white firm mass with internal whitish
mass with a mixed signal, ranging from dark to hyperin- calcific foci
11.3.2 Extraskeletal Osteosarcoma
a b
c d
e f
Fig. 11.4 Extraskeletal osteosarcoma. A patient under- ischium. The well-demarcated mass is isointense to mus-
went a total abdominal hysterectomy of a cervical cancer, cle on T1WI (c) and heterogeneously hyperintense on
followed by a course of radiation therapy 27years ago. T2WI (b, d). Postcontrast FS T1WI (e) shows heteroge-
She presented with left pelvic pain 3weeks ago. Pelvis neous enhancement with large non-enhancing areas.
radiograph (a) shows a multilobulated, multinodular Irregular-shaped dark signal area on all sequences repre-
dense calcification with some speculated margin adjacent sents the osteoid matrix mineralization. A photograph of
to left ischium. Coronal FS T2WI (b) shows a large soft the specimen (f) shows a yellowish mass with central
tissue mass in left ischiorectal fossa, medial to left large mineralization (star) and some hemorrhagic foci
References 191
a b
c d
Fig. 11.5 Extraskeletal osteosarcoma. MR image of the enhancement on postcontrast FS T1WI (c). A large irreg-
thigh reveals a circumscribed, large subcutaneous soft tis- ularly shaped non-enhancing hypointense component
sue with some infiltrative border (arrowhead). The tumor (arrows) suggests osteoid mineralization. A photograph
has heterogeneous hypo- to mild hyperintensity to muscle of the specimen (d) demonstrates whitish-yellow mass
on T1WI (a) and a mixed signal, ranging from dark to with eccentric mineralization (arrow) and focal hemor-
hyperintense on T2WI (b), with heterogeneous contrast rhagic foci
Kransdorf MJ, Meis JM.From the archives of the

References AFIP.Extraskeletal osseous and cartilaginous tumors
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tissue axillary mass in an adolescent. Case Rep mineralization of forearm extraskeletal chondroma.
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Reconstr Aesthet Surg. 2011;64(10):13779. Vaseenon T, Cheewawattanachai C, Pattamapaspong N,
doi:10.1016/j.bjps.2011.02.024. Settakorn J, Leerapun T.Extraskeletal chondroma on
Varma DG, Ayala AG, Guo SQ, Moulopoulos LA, the sole of the foot. Foot Ankle Spec. 2014;7(3):
Kim EE, Charnsangavej C.MRI of extraskeletal 2326. doi:10.1177/1938640013516790.
Nerve Sheath Tumors
12
Neurogenic neoplasms comprise 12% of all schwannoma affecting multiple nerve fascicles
benign and 78% of all malignant soft tissue or a nerve plexus, growing into thinly encapsu-
tumors. According to the 2013 WHO classifica- lated plexiform or multinodular tumors. Large
tion of tumors of soft tissue, the tumors dis- schwannomas with degenerative change, includ-
cussed in this chapter include schwannoma ing cyst formation, hemorrhage, calcification,
(including variants), neurofibroma (including hyalinization, or fibrosis, are often referred to as
variants), perineurioma, granular cell tumor, ancient schwannomas. These tumors affect all
malignant peripheral nerve sheath tumor, and ages, with a peak incidence in the 46th decade
malignant granular cell tumor. Other neuromas, of life; there is no sex predilection. These
such as traumatic or Mortons neuroma, are reac- schwannomas commonly arise in the peripheral
tive hyperplastic lesions and not true tumors; a nerves of the skin and subcutaneous tissue of the
complete discussion of these subtypes is there- head and neck or along the flexor surfaces of the
fore beyond the scope of this chapter. extremities. Ancient schwannomas usually pres-
ent as asymptomatic masses or incidental find-
ings on imaging studies. Multiple schwannomas
12.1 Schwannoma (Including may be associated with neurofibromatosis type 2
Variants) (accompanied by bilateral vestibular schwan-
noma, meningioma, or glioma) or schwannoma-
Schwannoma, also known as neurilemmoma or tosis (without vestibular schwannoma or
neurinoma, is a common, benign, usually encap- meningioma). The treatment of schwannoma is
sulated peripheral nerve sheath tumor (PNST) surgical resection. The affected nerve is fre-
composed of well-differentiated Schwann cells. quently separable from the tumor during the
This tumor has characteristic biphasic compo- operation, permitting postsurgical functionality
nents, with varying amounts of each. The first of of the native nerve. The lesion does not usually
these, Antoni A tissues, are compact areas of recur if treated by gross total resection. Malignant
spindle cells that show occasional palisading transformation is exceptionally rare (Antonescu
(Verocay bodies). Antoni B tissues are hypocel- etal. 2013b).
lular, less organized, more myxoid, and loosely Radiographs are frequently normal and occa-
arranged areas with lipid-laden histiocytes and sionally reveal a nonspecific soft tissue mass.
thick-walled, hyalinized blood vessels. Cellular Adjacent bone erosion or mineralizations are
schwannoma consists exclusively of Antoni A infrequently seen. On US and MR imaging, the
tissue. Plexiform schwannoma is defined as a entering and exiting nerve is occasionally

194 12 Nerve Sheath Tumors
detected in schwannomas that affect large, deep 12.2 Neurofibroma (Including

nerves (string sign); however, the nerve is dif- Variants)
ficult to identify in superficial or small lesions.
The tumors may appear fusiform, oriented along Neurofibroma is the most common benign
the long axis of the affected nerve. The lesions PNST, being composed of differentiated
may be located eccentrically to the affected Schwann cells, perineurial-like cells, fibro-
nerve with increasing tumor size. US examina- blasts, mast cells, and residual interspersed
tion demonstrates a well-defined homogeneous myelinated and unmyelinated axons embedded
or heterogeneous hypoechoic soft tissue mass in the extracellular matrix. This lesion mani-
with posterior acoustic enhancement. A target fests as one of the following five types: (1)
sign can be seen, characterized by a hypoechoic localized cutaneous, nodular or polypoid
peripheral zone and a hyperechoic central zone. lesions; (2) diffuse cutaneous, plaque-like
Hyperechoic calcifications and cystic formation lesions that extend into the subcutaneous tis-
or necrosis are occasionally observed in larger sue, with overlying hyperpigmentation; (3)
lesions. Doppler imaging reveals peripheral localized intraneural, solitary, segmental fusi-
blood flow signals that occasionally enter from form enlargements of sizeable nerves; (4)
the proximal and distal poles. On MR imaging, plexiform, intraneural, lumpy masses or worm-
schwannomas show a target sign consisting of a like growths that involve a nerve plexus or
central hypo- to isointensity and a peripheral multiple fascicles of a single nerve; and (5)
hyperintensity on T2-weighted images. These massive diffuse soft tissue plexiforms, ranging
characteristics represent histologically the cen- from a uniform regional soft tissue enlarge-
tral fibrocollagenous tissue and peripheral myx- ment to pendulous, bag-like, or cape-like
oid stroma. The lesions showing a target sign masses with widespread overlying skin hyper-
typically enhance more prominently at the cen- pigmentation. Most lesions occur sporadically
ter, with a surrounding area of non-enhancing as solitary lesions. Multiple or numerous neu-
myxoid tissue, effectively reversing the target rofibromas are observed in individuals with
sign. A fascicular sign is another characteristic neurofibromatosis (NF) type 1. All ages and
imaging finding, defined as multiple small round both sexes are affected. The lesions most com-
intermediate signal structures with a higher sig- monly affect the small nerves of the skin, fol-
nal intensity at the periphery on T2-weighted lowed by deeply located nerves of medium-size,
images. The split-fat sign is frequently observed nerve plexi or major nerve trunks. The treat-
as a rim of fat around the tumor, especially on ment is often surgical resection. In contrast to
T1-weighted images, representing the tumors schwannomas, neurofibroma tumor cells dif-
location in the intermuscular space around the fusely infiltrate the affected nerve. Complete
neurovascular bundle. The enhancement pattern excision of the tumor may therefore require
ranges from little enhancement, due to the abun- sacrifice of the affected nerve, leading to neu-
dant avascular myxoid tissue, to diffuse gradual rological deficits. Plexiform neurofibromas
enhancement. The tumor may occasionally be and localized intraneural neurofibromas are
accompanied by denervation changes, such as precursor lesions of malignant PNST.The
striated, increased intramuscular fat content or prevalence of malignant transformation varies
decreased muscle volume under the territory of from 2% to 29% in patients with NF type 1
the affected nerve. In larger lesions or ancient (Abreu etal. 2013; Antonescu etal. 2013a).
schwannomas, calcification, hemorrhage, The imaging findings of neurofibromas are
degenerative cyst formation, or necrosis may be similar to those observed with schwannomas,
noted on MR imaging (Abreu etal. 2013; as previously described, and in many cases,
Gruber etal. 2007; Banks 2005). these two entities cannot be distinguished. No
12.4 Granular Cell Tumor 195
single imaging feature or combination of fea- 12.3 Perineurioma

tures allows for definitive differentiation
between neurofibromas and schwannomas. Perineurioma is a rare benign peripheral nerve
When the parent nerve is identified, a centrally sheath tumor composed entirely of perineurial
located mass relative to the parent nerve, with cells. Two main types exist as follows: (1) the
an occasionally poorly defined or infiltrative rare intraneural type (also known as localized
nerve-tumor transition, suggests a neurofi- hypertrophic neuropathy), which is restricted to
broma. In contrast, as previously described, the peripheral nerve boundary, and (2) the more
eccentrically located masses suggest a schwan- common extraneural type, which commonly
noma (Ryu etal. 2015; Abreu etal. 2013; Tsai manifests in soft tissues and skin without con-
etal. 2008). A target sign is reported more necting to the peripheral nerves. Intraneural
commonly in neurofibromas than in schwanno- perineurioma primarily affects young adults and
mas, whereas, in our experience, the reverse children with accompanied motor deficiency
has been the case. The fascicular sign is and occasional sensory loss. Extraneural peri-
reported to be more common in schwannomas neurioma commonly arises in the subcutaneous
than in neurofibromas (Jee etal. 2004). tissue of the limb and trunk, with a peak inci-
Schwannomas are more commonly associated dence in middle-aged adults and usually pre-
with cyst formation, necrosis, and calcification senting as a painless mass (Macarenco etal.
than neurofibromas (Abreu etal. 2013). In 2007). Intraneural perineurioma is static or
terms of enhancement pattern, different reports slowly progressive, remaining confined to its
show inconsistent and contradictory results, original distribution and having a low morbid-
although the following signs have been pro- ity; it should therefore not be resected routinely
posed: Most neurofibromas are less vascular (Mauermann etal. 2009). Regarding extraneural
than schwannomas (Ryu etal. 2015); neurofi- perineurioma, the surgical resection is the treat-
bromas are frequently associated with central ment of choice.
enhancement, whereas schwannomas exhibit Intraneural perineurioma may demonstrate
diffuse enhancement (Jee etal. 2004); both characteristic MR imaging findings, including a
tumors are entirely hypervascular, with no sig- fusiform enlargement of the affected major nerve
nificant difference between them (Tsai etal. of considerable length. A hypo- or isointense
2008). Diffuse neurofibroma is frequently signal is observed on T1-weighted images, a
observed as a plaque-like or infiltrative lesion hyperintense signal on T2-weighted images, and
that involves the skin and subcutaneous tis- intense homogeneous enhancement. Extraneural
sues. In addition, this lesion commonly has perineurioma exhibits a circumscribed subcuta-
prominent internal vascularity and tends to neous or deep soft tissue lesion with hypointen-
spread along connective tissue septa, surround- sity on T1-weighted images, hyperintensity on
ing rather than destroying adjacent normal T2-weighted images, and inhomogeneous
structures (Hassell etal. 2008). Plexiform neu- enhancement, with a gradually enhancing nature
rofibroma has typical imaging findings, such as after contrast injection.
multiple nodules with a target sign along a
long nerve segment, nerve plexus, or its
branches. This pattern results in a characteris- 12.4 Granular Cell Tumor
tic serpentine bag of worms appearance. In
addition, diffuse neurofibromas may exhibit Granular cell tumor (GrCT) is a rare benign tumor
ill-defined margins due to their subcutaneous that exhibits neuroectodermal differentiation and
spread along connective tissue septa (Murphey is composed of large, oval to round cells with
etal. 1999). copious eosinophilic granular cytoplasm. GrCT
commonly affects adults in the 46th decade of 12.5 M

alignant Peripheral Nerve
life, with a male predominance. This lesion occurs Sheath Tumor
frequently in the head and neck, including the
tongue, followed by chest wall, breast, back, and Malignant peripheral nerve sheath tumor
extremities. This tumor commonly affects the (MPNST) is a rare, highly aggressive tumor aris-
skin/subcutis, submucosa, and uncommonly deep ing either from a peripheral nerve, from a pre-
soft tissue, such as the muscle. GrCT presents as a existing benign PNST (usually neurofibroma), or
painless mass or is asymptomatic (Lazar 2013; from neurofibromatosis type 1 (NF-1) (Nielsen
Billeret Lebranchu 1999; Lack etal. 1980). etal. 2013). MPNSTs account for 310% of soft
Radiography shows a nonspecific soft tissue tissue sarcomas and are diagnosed most com-
swelling. US may demonstrate a circumscribed or monly in patients aged between 20 and 50years.
irregularly bordered, heterogeneous hypoechoic Patients with NF-1 present with MPNST less
soft tissue mass in the subcutaneous tissue or mus- than 10years earlier than those patients with non-
cle. This mass may be accompanied by a thin echo- NF-1 MPNST (Grobmyer etal. 2008). MPNST
genic rim with poorly defined fuzzy margins, presents as an enlarging mass or unexplained
occasional posterior sonic enhancement, tiny inter- change in size or the development of new symp-
nal calcifications, and focal vascularity on color toms in NF-1 patients. Malignant PNST can also
Doppler imaging (Kim etal. 2016; Kim etal. 2011; be a secondary neoplasm related to previous radi-
Gruber etal. 2007). On MR imaging, GrCT is ation therapy. Such tumors develop after a long
observed as a well-defined or ill-defined round or latent period (1020years) following irradiation
oval soft tissue mass in the subcutaneous tissue or (Wanebo etal. 1993). The treatment of MPNST
muscle. Isointensity or mild hyperintensity relative is complete surgical excision with wide resection
to the muscle is observed on T1-weighted images, margins. Despite this aggressive treatment, local
and peripheral hyperintensity and central hypo- to recurrence and distant metastases are common
isointensity relative to the muscle are observed on (Grobmyer etal. 2008).
T2-weighted images. The contrast enhancement No imaging criteria can reliably differenti-
pattern is variable. Intratumoral hypointense areas ate malignant from benign PNSTs, but some
on T1- and T2-weighted images may be attribut- imaging features could be useful for the differ-
able to a fibrous/collagenous component or possi- ential diagnosis of MPNST, as follows: periph-
bly a ribbonlike or trabecular arrangement of tumor eral enhancement with non-cystic appearance
cells (Blacksin etal. 2005). One author reported a or remarkable heterogeneous enhancement,
stripe sign of GrCT on US and MR imaging, large tumor (>5cm), ill-defined margins, inva-
observed as hypointense intratumoral lines that are sion of fat planes, perilesional edema, intratu-
continuous with the adjacent muscle fibers at the moral lobulation, absence of a target sign, and
craniocaudal portion of the mass, representing bone destruction (Yu etal. 2016; Abreu etal.
preservation of the adjacent muscle fibers (Kim 2013; Pilavaki etal. 2004; Murphey etal.
etal. 2016). 1999).
12.6 Illustrations: Nerve Sheath Tumors
12.6.1 Schwannoma
a b
c d
Fig. 12.1 Schwannoma. Axial T1WI of the pelvis (a) non-enhancing myxoid tissue. A split-fat sign (arrow-
shows a circumscribed oval soft tissue mass within the heads) observed as a rim of fat around the tumor is noted
gluteus maximus muscle, with a mild central hyperinten- on coronal T1WI (d). A photograph of the specimen (e)
sity and peripheral hypointensity to muscle. T2WI (b) shows encapsulated soft tissue mass consisting of central
reveals a target sign consisting of central hypo/isointen- white/yellowish cellular or fibrocollagenous areas or
sity and peripheral bright hyperintensity, whereas post- patches of hemorrhage and peripheral, translucent, glis-
contrast FS T1WI (c) reveals a reverse target sign tening myxoid tissue
composed of central intense enhancement and peripheral
a b
c d
Fig. 12.2 Schwannoma. Sagittal T1WI (a) and T2WI (b) cular mass with a target sign composed of a heteroge-
of the knee show a circumscribed, multilobulated, fusi- neous hyperechoic central area and a homogeneous
form soft tissue mass within the lateral head of the gas- anechoic to hypoechoic periphery. The lesion has poste-
trocnemius muscle. The lesion is isointense to muscle on rior sonic enhancement and some peripheral vascularity
T1WI.On T2WI (b, c), the mass shows central multilobu- on color Doppler image (f). The entering/exiting nerve is
lated hypointensity and peripheral fluid-equivalent bright not depicted on US.A photograph of the specimen (g)
hyperintensity, generating the target sign. A split-fat sign shows a well-circumscribed mass consisting of central
(arrowheads) is observed as fat around the tumor. Axial white/yellowish cellular or fibrocollagenous areas with
postcontrast FS T1WI (d) reveals central intense enhance- some internal hemorrhaging and peripheral, translucent,
ment and peripheral sparse enhancement, reversing the glistening yellow myxoid tissue
target sign. US (e) demonstrates well-bordered intramus-
e g
a b
c d
Fig. 12.3 Schwannoma. Axial T1WI (a) and T2WI (b) is located eccentrically to the mass. Longitudinal US (d)
of the forearm show a circumscribed, oval, intermuscular reveals a well-demarcated, fusiform, heterogeneous
soft tissue mass along the ulnar nerve. The lesion is isoin- hypoechoic mass with posterior sonic enhancement. The
tense to muscle on T1WI and heterogeneous hyperinten- ulnar nerve (small arrow) is located eccentrically to the
sity on T2WI, with inhomogeneous mild enhancement on mass. Intralesional vascularity is absent on color Doppler
postcontrast FS T1WI (c). The ulnar nerve (small arrow) image (not shown)
a b
c d
Fig. 12.4Schwannoma with cystic degeneration. T1WI (c) shows heterogeneous peripheral and septal
Sagittal FS T2WI (a) of the distal thigh shows a circum- enhancement, with multifocal non-enhancing cystic or
scribed soft tissue mass arising from the distal sciatic hemorrhagic necrotic areas. US (d) reveals the heteroge-
nerve (arrowhead). The mass has a heterogeneous signal neous echogenic soft tissue mass, with a signal ranging
on T2WI, ranging from a fluid-fluid level (representing from anechoic to hyperechoic, with posterior sonic
hemorrhage) to bright hyperintensity. On axial T1WI (b), enhancement. A biopsy was performed to target the solid
the mass has an inhomogeneous isointense to mildly component of the mass
hyperintense signal (to muscle). Axial postcontrast FS
12.6.2 Cellular Schwannoma
a b
c d
Fig. 12.5 Cellular schwannoma. Axial T1WI of the cal non-enhancing foci (small arrows), and is slightly
upper arm (a) reveals an oval mass, slightly hyperintense hyperintense on T1WI (a), representing hemorrhagic
to muscle, located along the path of the ulnar nerve. T2WI areas. A string sign is noted on sagittal FS T2WI (d) in
(b) reveals a fascicular sign, observed as multiple small which the entering and exiting nerve (stars) is visualized
round intermediate signal structures with peripheral at both ends of a vertically oriented mass. Sectioned sur-
higher signal intensity. The tumor has heterogeneous face of the specimen (e) reveals a firm, yellowish mass
enhancement on postcontrast FS T1WI (c), with multifo- with spotty hemorrhagic foci
12.6.3 Plexiform Schwannoma
a b
c d
Fig. 12.6 Plexiform schwannoma. Coronal FS (a) and tense rim, respectively. Sequentially cut surfaces of the
axial (b) T2WIs of the knee show aggregation of variably specimen (c) reveal multiple, well-encapsulated masses
sized, circumscribed soft tissue lesions affecting the skin and a firm yellowish surface. Brain MR image (d) of the
and subcutaneous tissue, which are represented by a cen- same patient shows bilateral vestibular schwannomas
tral intermediate signal area and a thin peripheral hyperin- (arrows), representing neurofibromatosis type 2
a d
Fig. 12.7 Plexiform schwannoma. Axial T1WI of the enhancement on postcontrast FS T1WI (c). Marked, cord-
hand (a) shows a deep subcutaneous multinodular iso- to like, diffuse enlargement of the affected palmar digital
mildly hyperintense mass in the palm. The tumor is nerve of the index finger is observed on coronal FS T2WI
hyperintense on T2WI (b) and exhibits gradual diffuse (d)
12.6.4 Ancient Schwannoma
a b
c d e
Fig. 12.8 Ancient schwannoma. Radiograph of the thigh enhancement and some non-enhancing areas are observed
(a) shows a circumscribed soft tissue mass with internal on postcontrast FS T1WI (e). A photograph of the speci-
spotty calcifications (small arrows). The tumor is eccen- men (f) reveals many hemorrhagic areas and central hya-
trically located along the sciatic nerve (stars), with promi- linization. The non-enhancing peripheral hyperintense
nent peritumoral fat overgrowth on coronal T2WI (b). rim (arrowheads) on T2WI (b, d) corresponds to myxoid
The lesion is inhomogeneously hyperintense to muscle on tissue
T1WI (c) and mixed signal on T2WI (d). Heterogeneous
a b
c d
Fig. 12.9 Ancient schwannoma. Coronal T1WI (a) of some septal/solid enhancement on postcontrast FS T1WI
the thigh shows a well-defined, deep subcutaneous (c). A photograph of the specimen (d) reveals a large cyst
hypointense oval mass. The tumor has fluid-equivalent with upper, mixed smaller cystic, and myxoid solid areas
hyperintensity on T2WI (b), with a peripheral rim and
a b
c d
e f
Fig. 12.10 Ancient schwannoma. Coronal T1WI (a) of Contrast CT (e) reveals a well-defined heterogeneous
the shoulder shows a circumscribed large hypointense low-attenuation mass with an internal fuzzy enhancing
mass adjacent to the brachial plexus. The mass has hetero- area and thin peripheral rim enhancement. Intratumoral
geneous hyperintensity with multifocal hypointense foci calcification is not depicted on CT.Photograph of a
on T2WI (b) and heterogeneous enhancement with a large sequentially cut surface (f) reveals a large myxoid mass
central non-enhancing area on postcontrast FS T1WI (c). with an internal hemorrhagic area and calcific foci
PET (d) shows the mass to be mildly hypermetabolic.
12.6.5 Neurofibroma
a b
c d
Fig. 12.11 Neurofibroma. Axial T1WI (a) of the thigh delayed gradual mild enhancement on axial postcontrast
reveals a circumscribed mass with central mild hyperin- FS T1WI (d). Focal central signal voids on all sequences
tensity to dark signal within the biceps femoris muscle. suggest hemosiderin deposit. A photograph of the speci-
The tumor shows central hypointensity and peripheral men (e) shows central whitish-yellow solid area with
hyperintensity, so-called, target sign on T2WI (b). Sagittal internal large hemorrhagic component, and surrounding
postcontrast FS T1WI (c) reveals central enhancing area semitranslucent glistening myxoid tissue
and surrounding non-enhancing tissue, which shows
a d
e f
Fig. 12.12 Neurofibroma. MR images of the thigh reveal (d) reveals a string sign with a central location to the
a circumscribed fusiform mass within the sartorius mus- nerve (arrowheads). On US (e), the tumor has a smooth
cle. The tumor is isointense to muscle on T1WI (a) and surface and lobulation, with heterogeneous isoecho-
hyperintense on T2WI (b), with fuzzy inhomogeneous genicity to muscle. The sectioned surface (f) is smooth,
enhancement on postcontrast FS T1WI (c). Sagittal T2WI glistening, semitranslucent, and firm
a b
c d
Fig. 12.13 Neurofibroma, diffuse type. Sagittal (a) and hyperintense with intralesional tortuous flow voids and
axial (c) T1WIs show a very large plaque-like, infiltrating, multiple thin, linear hypointensities. Axial postcontrast
inhomogeneous isointense mass in the subcutaneous fat FS T1WI (d) shows heterogeneous enhancement. The cut
of the foot dorsum, extending from the skin to the fascia. surface (e) is variably whitish-ivory in color, with scat-
Sagittal FS T2WI (b) shows the mass to be generally tered dark brown or brown hemorrhagic foci
a b
c d
Fig. 12.14 Neurofibroma, diffuse type. Radiograph (a) neous mass show heterogeneous isointensity on coronal
shows increased soft tissue around the middle finger in T1WI (b) and heterogeneous hyperintensity on T2WI (c).
addition to abnormal elongation, bone pressure erosions, Inhomogeneous intense enhancement is observed on post-
and some proliferation. The infiltrating skin and subcuta- contrast FS T1WI (d)
12.6.6 Extraneural Perineurioma
a b
c d
Fig. 12.15 Extraneural perineurioma. Sagittal T1WI (a) The lesion has intermediate signal on T2WI (b) and
of the knee reveals a small, circumscribed, periarticular homogeneous contrast enhancement on postcontrast FS
soft tissue nodule that is isointense to muscle. The mass is T1WI (c). Axial FS PDWI (d) shows the juxta-articular
located posterior to the posterior lateral femoral condyle. location of the small hyperintense nodule
12.6.7 Intraneural Perineurioma
a b
c d
Fig. 12.16 Intraneural perineurioma. Axial T1WI of the particularly severe involvement of the distal half. Note the
thigh (a) shows a mild isointense swelling of the sciatic extensive and infiltrative soft tissue change in the inter-
nerve (arrow). The affected sciatic nerve has an interme- muscular fat plane around the nerve (arrowheads). An
diate signal on T2WI (b) and heterogeneous enhancement enlarged sciatic nerve is observed on US (e), with relative
on postcontrast FS T1WI (c), with preservation of the preservation of nerve fascicular structures and hyper-
internal fascicular appearance. Sagittal FS T2WI (d) dem- echoic perineural soft tissue hypertrophy (star)
onstrates diffuse involvement of the sciatic nerve, with
12.6.8 Granular Cell Tumor
a b
c d
e f
Fig. 12.17 Granular cell tumor. MR images of the thigh hyperintensity. Intratumoral dark signal foci (small
show an irregular, speculated bordered soft tissue mass arrows) on all sequences represent a fibrocollagenous
within the vastus medialis muscle. The lesion is isointense component. US (e) on the transverse plane reveals an ill-
to muscle on T1WI (a), central heterogeneous hypo/isoin- defined heterogeneous hypoechoic mass with a surround-
tensity and peripheral hyperintensity on T2WI (b), and ing, irregular hyperechoic rim. A photograph of the
inhomogeneous enhancement on postcontrast FS T1WI specimen (f) shows an irregularly bordered whitish firm
(c). Coronal FS T2WI (d) reveals an intermediate signal mass without hemorrhage or cyst/necrosis
ovoid mass with irregularly marginated, peripheral T2
a b
c d
Fig. 12.18 Granular cell tumor. Axial T1WI of the fore- mild mass effect on adjacent muscle without direct tumor
arm (a) reveals an irregularly bordered iso- to slightly extension. US reveals heterogeneous hypoechoic subcuta-
hyperintense (relative to muscle) subcutaneous mass, neous mass with perilesional thin hyperechoic rim and
based on the deep fascia. The tumor shows inhomoge- mild intratumoral hypervascularity on color Doppler
neous hyperintensity on FS T2WI (b) and intense image (e)
enhancement on postcontrast FS T1WI (c). The tumor has
12.6.9 Malignant Granular Cell Tumor
a b
c d
Fig. 12.19 Malignant granular cell tumor. Axial T1WI (c), with more intense peripheral rim enhancement. US
of the pelvis (a) shows a circumscribed, ovoid, isointense (d) reveals a circumscribed, homogeneously hypoechoic
(to muscle) mass within the gluteus maximus muscle, intramuscular mass. A photograph of the specimen (e)
with a split-fat sign (small arrow). The mass shows inho- shows a thinly encapsulated, yellowish mass with some
mogeneous intermediate signal on T2WI (b) and hetero- myxoid change and no hemorrhaging or necrosis
geneous enhancement on coronal postcontrast FS T1WI
12.6.10 M
alignant Peripheral Nerve
Sheath Tumor
a b
c d
Fig. 12.20 Malignant peripheral nerve sheath tumor. nal non-enhancing area are observed on postcontrast FS
Axial T1WI of the lower leg (a) shows an ill-defined, T1WI (c). Longitudinal US (d) reveals a heterogeneous
deep-seated, mildly hyperintense soft tissue mass (arrow) hypoechoic mass with some infiltrative borders (arrow-
beneath the crural fascia. The lesion has heterogeneous heads), mild posterior sonic enhancement, and an enter-
hyperintensity on T2WI (b). Heterogeneous contrast ing/exiting nerve. Mild perilesional vascularity is
enhancement with an infiltrative border and a focal, inter- observed on the color Doppler images (not shown)
a b
c d e
Fig. 12.21 Malignant peripheral nerve sheath tumor. (arrow). The mass is isointense to muscle on T1WI (c)
Sagittal FS T2WI (a) of the lower leg in a patient with and is heterogeneously hyperintense on T2WI (d), with
NF-1 shows a typical appearance: a diffuse thickened inhomogeneous enhancement on postcontrast FS T1WI
nerve with nodular plexiform neurofibroma affecting the (e). PET (f) shows the mass to have higher metabolism
common peroneal nerve and its branches. The target sign (SUV 3.3) (arrow) compared with other neurofibromas.
with low signal centrally and high signal intensity periph- On US (g), the mass has heterogeneous hypoechogenicity
erally is observed in numerous lesions. Two years later, with prominent internal vascularity on the color Doppler
the patient complained of local pain and swelling at the image (h). Photograph of the sequentially cut surface (i)
proximal tibial level. Follow-up sagittal T2WI (b) shows reveals a large firm yellowish mass with an internal curvi-
internal development of a large subcutaneous mass linear hemorrhagic area
f g
a b c
d e
Fig. 12.22 Malignant peripheral nerve sheath tumor. T1WI (c). A central, mildly T1 hyperintense, T2 interme-
Coronal MR images of the thigh in a patient with NF-1 diate, non-enhancing area is suggestive of hemorrhage.
show a diffuse thickened and nodular sciatic nerve with a PET (d) shows the mass to be markedly hypermetabolic
bag of worms appearance (arrowheads), representing (SUV 18.5) compared with other neurofibromas. A photo-
plexiform neurofibroma. The large mass eccentric to the graph of the specimen (e) reveals a large, multinodular,
sciatic nerve is depicted with inhomogeneous isointense firm whitish mass with an internal hemorrhagic area aris-
signal (to muscle) on T1WI (a), heterogeneous signal ing from the underlying plexiform neurofibroma
(ranging from hypo- to hyperintensity) on T2WI (b), and (arrowheads)
heterogeneous intense enhancement on postcontrast FS
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Tumors ofUncertain
Differentiation 13
Tumors of uncertain differentiation include many mass without pain or tenderness. The age of pre-
soft tissue tumors that have an unclear line of cel- sentation is 4070years of age, with a female
lular differentiation. In some tumors (e.g., mixed predilection (Zou etal. 2013). The most frequent
tumors, synovial and clear cell sarcomas), a line sites of these tumors are the intramuscular com-
of differentiation can be identified, but a cellular partments of the thigh (51%), upper arm (9%),
counterpart of the tumor cannot be defined in calf (7%), or buttock (7%) (Murphey etal. 2002).
normal mesenchymal tissues (Kransdorf 2014). The coexistence of multiple myxomas and fibrous
There were some minor changes in the latest dysplasia of the bone, generally in the same ana-
version of the WHO classification (Fletcher etal. tomic region, is referred to as Mazabraud syn-
2013); specifically, two tumors were added to this drome (Kabukcuoglu etal. 2004; Delabrousse
category. The first of these is hemosiderotic etal. 2001; Perez Sanchez and Gonzalez Llorente
fibrolipomatous tumor (HFLT), which is a locally 2014).
aggressive neoplasm that commonly arises around Imaging findings of myxoma are similar to
the ankle or wrist. The second addition to this cat- those of a cyst on CT and MR imaging, showing
egory is phosphaturic mesenchymal tumor a lesion composed of a large amount of mucin
(PMT). In addition, in the 2013 WHO classifica- and a low collagen content, as observed histo-
tion, the term primitive neuroectodermal tumor logically (Murphey etal. 2002). Myxoma is a
(PNET) has been removed as a synonym for well-circumscribed, homogeneous intramuscular
Ewing sarcoma with a variable degree of neuronal mass with very high signal intensity on
differentiation. This change was made to mini- T2-weighted MR images, quite similar to the sig-
mize confusion with PNET of the central nervous nal characteristic of fluid (Murphey etal. 2002;
system and female genital tract, which is histo- Yao etal. 2007; Bancroft etal. 2002). This lesion
logically and genetically different from PNET in shows a variable degree of contrast enhancement
the extremities (Fletcher etal. 2013). proportional to the amount of solid myxoid tissue
and fibrous septa within the tumor, allowing it to
be differentiated from cystic lesions. US shows a
13.1 Intramuscular Myxoma well-defined, heterogeneous hypoechoic mass
surrounded by normal muscle, occasionally
Intramuscular myxoma is a benign mesenchymal accompanied by small, fluid-filled clefts and cys-
lesion composed of spindle- to stellate-shaped tic areas. The detection of internal echoes of the
fibroblasts, paucicellular myxoid matrix, and mass is useful in the differentiation of a solid
sparse blood vessels (Luna etal. 2005). Patients mass from a cystic lesion. A sonographic bright
usually present with a slowly growing palpable rim sign of echogenicity around the mass may

222 13 Tumors ofUncertain Differentiation
reflect increased fat deposition around intramus- Unlike other soft tissue sarcomas, eccentric or
cular myxoma (Girish etal. 2006; Kim 2014). On peripheral mineralization is identified within the
color Doppler study, these lesions often show mass in approximately 2030% of cases on radi-
little or no vascularity due to a paucity of vascu- ography (Murphey etal. 1999). CT is useful for
lar structures within the tumor. Mucoid material detecting calcification and bone involvement in
may leak into the immediately adjacent muscle, synovial sarcoma (Murphey etal. 2006; Vliet
which could lead to muscle atrophy with sur- etal. 2009). On T2-weighted MR images, syno-
rounding fat deposition and/or muscle edema. vial sarcoma has a markedly heterogeneous sig-
These histologic findings correspond to presence nal intensity pattern that is referred to as a triple
of a small rim of fat and/or surrounding edema on sign, being characterized by intermixed areas of
MR imaging and a bright rim sign of echogenicity low (dystrophic calcifications and fibrotic bands),
around the mass on ultrasonography (Bancroft intermediate (soft tissue components), and high
etal. 2002; Girish etal. 2006; Murphey etal. signal intensity (necrosis and hemorrhage)
2002). (Murphey etal. 2006; Vliet etal. 2009). There
may be areas of hemorrhage with fluid-fluid lev-
els in up to 1025% of the tumors, a sign that is
13.2 Synovial Sarcoma known as a bowl of grapes (Jones etal. 1993;
Murphey etal. 2006; Vliet etal. 2009). Lesions
Synovial sarcoma is a relatively common soft tis- smaller than 5cm are often superficial and fre-
sue sarcoma in adults, accounting for approxi- quently well-defined, mimicking benign pro-
mately 510% of soft tissue sarcomas (Kransdorf cesses in terms of homogeneous signal
1995; Kempson etal. 2001). Synovial sarcoma characteristics and a lack of invasion into adja-
typically occurs in adolescents and young adults cent structures (Jones etal. 1993; Murphey etal.
between 15 and 35years of age. Patients with 2006; Vliet etal. 2009). Contrast enhancement is
synovial sarcoma usually present with a palpable, usually prominent and can be heterogeneous or
slowly growing soft tissue mass that may give a homogeneous. Dynamic contrast-enhanced MR
false impression of a benign process, and there is images (DCE-MRI) show a rapid progressive lin-
often a delay in diagnosis. ear increase in signal intensity followed by a
Synovial sarcoma is a misnomer because it washout or plateau in 60% of cases (Van Rijswijk
does not arise from or differentiate toward the etal. 2001). Several MR features have been
synovium. Synovial sarcoma is thought to origi- reported as prognostic parameters associated
nate from primitive (uncommitted) mesenchymal with high tumor grade and a less favorable prog-
cells with variable degrees of epithelial differentia- nosis. Imaging findings favoring a diagnosis of
tion. Histologically, synovial sarcoma is composed high-grade tumor included proximal distribution,
of two morphologically different cell types (epi- large tumor size, the absence of calcifications, the
thelial and spindle cells) and can be classified into presence of cysts, the presence of hemorrhage,
three subtypes depending on cell type composition and the presence of a triple signal pattern (Tateishi
and differentiation. The biphasic type consists of etal. 2004).
spindle cells and epithelial cells (usually forming
glands). The monophasic type is predominantly
made up of mesenchymal spindle cells and is rela- 13.3 Phosphaturic Mesenchymal
tively common. The poorly differentiated type Tumor
consists of cells that are generally epithelioid in
morphology and have high mitotic activity; the Phosphaturic mesenchymal tumor (PMT) is a rare
clinical behavior of this type is more aggressive soft tissue or bone tumor that is often associated
(Murphey etal. 2006; Vliet etal. 2009; Bakri etal. with a paraneoplastic syndrome known as tumor-
2012). A specific chromosomal translocation induced (oncogenic) osteomalacia (TIO). The
t(X;18)(p11;q11) has been reported in association tumor appears to overexpress fibroblast growth
with synovial sarcoma (Murphey etal. 2006). factor 23 (FGF-23), which inhibits renal tubular
13.4 Epithelioid Sarcoma 223
epithelial phosphate transport, resulting in osteo- 13.4 Epithelioid Sarcoma

malacia (Folpe etal. 2004). Previously, these
tumors were considered a heterogeneous group of Epithelioid sarcoma typically affects adolescents
bone and soft tissue tumors, including hemangio- and young adults between 10 and 35years of age
pericytoma, hemangioma, giant cell tumor, or (Hanna etal. 2002). There are two principal types
osteoblastoma. However, it has been recognized of epithelioid sarcoma as follows: the conven-
by certain investigators that many of these tumors tional (classical or distal) type and the proximal
are, in fact, quite distinctive when carefully evalu- type (Guillou etal. 1997; Chbani etal. 2009).
ated (Folpe etal. 2004). In 1987, Weidner and Conventional-type epithelioid sarcoma most
Santa Cruz used the term phosphaturic mesen- commonly arises on the flexor surfaces of the fin-
chymal tumor, mixed connective tissue type gers, hands, and forearm, followed by the knee/
(PMTMCT) to describe these unique lesions, lower leg and the buttocks/thigh (Chase and
which are characterized by a distinctive admix- Enzinger 1985). The proximal-type epithelioid
ture of spindled cells, osteoclast-like giant cells, sarcoma has a predilection for arising in axial
microcysts, prominent blood vessels, cartilage- locations (e.g., perineum, pelvis, vulva, and but-
like matrix, and bone (Weidner and Santa Cruz tocks) and more aggressive behavior (Tateishi
1987). The authors divided PMTs into the follow- etal. 2002). The tumor occurs in both the super-
ing four subtypes based on histology: (1) mixed ficial and deep soft tissues. When located superfi-
connective tissue tumors (PMTMCT); (2) osteo- cially, it usually presents as a firm nodule that
blastoma-like tumors; (3) non-ossifying fibroma- may be solitary or multiple. Superficial lesions
like tumors; and (4) ossifying fibroma-like are often elevated above the skin surface and
tumors. PMTMCT is the most common type, become ulcerated in 10% of cases. Deep-seated
accounting for over 90% of TIO-associated mes- lesions are usually firmly attached to tendons,
enchymal tumors (Weidner and Santa Cruz 1987; tendon sheaths, or fascial structures, being infil-
Folpe etal. 2004). trative and heterogeneous in appearance (Hanna
PMT often occurs in middle-aged adults in the etal. 2002).
soft tissue and bone. In many cases, the patients Radiograph or CT can reveal calcification or,
presented with a long-standing history of osteo- rarely, ossification in 828% of cases (Lo etal.
malacia, generalized fatigue, and pain with mul- 1977; Tateishi etal. 2002). The MR features are
tiple pathologic fractures. Those nonspecific generally nonspecific. The tumor may present as
symptoms and a vague clinical presentation often infiltrative intramuscular masses or as cutaneous
result in a long delay in the diagnosis and treat- nodules. Large lesions can exhibit an inhomoge-
ment of patients with PMT.Once the diagnosis of neous signal pattern on T2-weighted images due
PMT is considered, tumor localization is the next to the presence of hemorrhage or necrosis. The
challenging issue due to their variable location tumor is usually isointense to the muscle on
and small size. Compartmental venous sampling T1-weighted images but occasionally
of FGF-23 has been useful inlocalization of the demonstrates high signal intensity, which corre-
body region in which the tumor is located. Then, sponds to areas of hemorrhagic necrosis. A peri-
MR imaging of the body region can be performed tumoral edema-like signal that affects the
to identify the lesion. Octreotide scanning is surrounding muscles is observed in nearly 70%
another helpful imaging technique for localizing of cases and histologically corresponds to intense
these small tumors because they may express inflammation (Hanna etal. 2002). The tumor
somatostatin receptors. may demonstrate multiple intrinsic hypointense
Although most PMTs are histologically and foci on both T1- and T2-weighted images, corre-
clinically benign, surgical resection of the caus- sponding to abundant soft tissue calcifications.
ative tumor is the treatment of choice to com- Other common findings include tumor encase-
pletely resolve the symptoms. The prognosis of ment of the adjacent neurovascular bundle
PMT is generally good, but rare metastatic cases and enlarged regional lymph nodes (Hanna etal.
have been described (Ogose etal. 2001). 2002). Although the tumor has nonspecific
imaging findings, suspicion for epithelioid sar- d emonstrates a soft tissue mass with isoattenua-
coma should arise in patients presenting with tion to that of the adjacent muscle. After contrast
multiple soft tissue nodules or persistent punched- administration, the mass enhances markedly with
out ulcers involving the skin and subcutaneous prominent feeding arteries and draining veins
tissues, particularly of the extremities (Hanna (Tian etal. 2016). On MR imaging, this tumor
etal. 2002). Epithelioid sarcoma is notorious for typically shows an equal or hyperintense signal
multiple recurrences and high propensity to intensity on T1-weighted images and a heteroge-
metastasize to regional lymph nodes and the lung neous high signal intensity on T2-weighted
(Chase and Enzinger 1985; Hanna etal. 2002). images, with multiple flow voids. Serpentine flow
voids can be observed at the margin of the lesion
and within the mass. After contrast medium
13.5 Alveolar Soft Part Sarcoma administration, intense enhancement and tortuous
and dilated vessels can be observed in and around
Alveolar soft part sarcoma is a relatively rare the mass. Although arteriovenous malformation
malignant soft tissue tumor, constituting fewer (AVM) could be considered a differential diagno-
than 1% of all soft tissue sarcomas. This tumor sis, AVM differs from alveolar soft part sarcoma
was named by Christopherson etal., who based on a rapid washout of contrast on dynamic
observed the cells to have a characteristic pseu- contrast-enhanced MR images and a lack of a soft
doalveolar or organoid arrangement formed by tissue component (Suh etal. 2000; McCarville
aggregates of large granular cells surrounded by etal. 2014; Tian etal. 2016).
vascular channels that mimic the pattern of respi-
ratory alveoli (Christopherson etal. 1952).
Alveolar soft part sarcoma occurs principally 13.6 Clear Cell Sarcoma
in adolescents and young adults and is most fre-
quently observed in the thigh or buttock Clear cell sarcoma predominantly affects young
(Lieberman etal. 1966; Lieberman etal. 1989; adults between the ages of 20 and 40years
Portera etal. 2001; Anderson etal. 2005; (Goldblum etal. 2014). The patient presents with
Pennacchioli etal. 2010). When the tumor affects a slowly growing mass, which causes pain or ten-
infants and children, it is often located in the derness in nearly half of cases. In the past, this
region of the head and neck, especially the orbit tumor has been referred to as malignant mela-
and tongue (Casanova etal. 2000; Orbach etal. noma of soft parts due to its histologic similari-
2013). It usually presents as a slowly growing, ties to malignant melanoma (Chung and Enzinger
painless mass. The mass may be pulsatile with an 1983; Lucas etal. 1992; Graadt van Roggen etal.
associated bruit. Although the tumor is slow 1998). Intracellular melanin can be identified in
growing, it has a high rate of metastasis, which is 72% of cases (Chung and Enzinger 1983, 163).
thought to be due to the extreme vascularity of However, clear cell sarcoma is clinically and bio-
the tumor and its early microvascular invasion logically distinct from cutaneous melanoma.
(Anderson etal. 2005). The common sites of This tumor primarily arises in the deep soft tis-
metastases are the lung, followed by the brain sues of the lower extremities (foot, ankle, knee
and bone. and thigh), adjacent to tendons, aponeuroses, and
Radiographs may demonstrate a nonspecific fascial structures (Lucas etal. 1992). Most of the
soft tissue mass, but punctate calcification may be tumor has a consistent balanced translocation
observed (Lorigan etal. 1989). Alveolar soft part t(2;22)(q13;q12) that can help distinguish it from
sarcoma is so hypervascular that angiography cutaneous malignant melanoma (Reeves etal.
reveals hypervascularity with prominent draining 1992; Rodriguez etal. 1992; Langezaal etal.
veins and prolonged capillary staining (Suh etal. 2001; Segal etal. 2003).
2000; McCarville etal. 2014; Tian etal. 2016). Radiographs do not contribute to the diagnosis
Ultrasonography may be useful to identify the and may only show the nonspecific soft tissue
hypervascular nature of the tumor. CT mass. On MR imaging, clear cell sarcomas usually
13.8 Extraskeletal Ewing Sarcoma 225
present as well-circumscribed lesions with vari- tion on CT and very high signal intensity on
able signal intensity on T1-weighted and T2-weighted MR images, indicating its extremely
T2-weighted images.Approximately 50% of high water content. Lobules of very high signal
tumors have slightly increased signal intensity on intensity may be defined by thin fibrous septa of
T1-weighted images compared with the muscle low signal intensity on T2-weighted image. On
and display melanocytic differentiation (De T1-weighted images, the tumor is heterogeneously
Beuckeleer etal. 2000; Hourani etal. 2005). The isointense to the muscle and frequently shows
paramagnetic effect of intratumoral melanin hyperintense foci that correspond to hemorrhagic
causes a shortening of T1 relaxation time, lead- changes within the tumor. After intravenous con-
ing to increased signal intensity on T1-weighted trast administration, the tumor also shows periph-
images. As T1 hyperintensity is rarely observed eral/septal enhancement, as observed in a
in soft tissue tumors, this characteristic MR chondroid tumor (Gebhardt etal. 1999; Tateishi
image helps to narrow the list of differential diag- etal. 2006; Kapoor etal. 2014).
noses (De Beuckeleer etal. 2000; Hourani etal.
2005). After intravenous contrast administration,
strong enhancement is observed in most cases. 13.8 Extraskeletal Ewing Sarcoma
As clear cell sarcoma has a benign- looking
appearance on MR imaging, the diagnosis should Extraskeletal Ewing sarcoma usually manifests in
be suspected when the lesion is a well-defined, young patients between 10 and 30years of age.
homogeneous, strongly enhancing mass with a Males are affected slightly more commonly than
slightly higher signal intensity compared with the females. Clinically, patients often have a large, rap-
muscle on T1-weighted images. idly growing, solitary, superficial, or deep soft tissue
mass (Murphey etal. 2013). The most commonly
reported locations of extraskeletal Ewing sarcoma
13.7 Extraskeletal Myxoid include the paravertebral region (32%), lower
Chondrosarcoma extremities (26%), chest wall (18%), retroperito-
neum (11%), pelvis and hip (11%), and upper
Extraskeletal myxoid chondrosarcomas typically extremities (3%) (Murphey etal. 2013).
occur in the extremities, with a predilection for the Histologically, the tumor has monotonous prolifera-
thigh. It commonly affects patients aged 5060years tion of solidly packed small blue round cells with no
but is also known to occur in younger people differentiating features. Necrosis, cystic changes,
(Enzinger and Shiraki 1972; Kapoor etal. 2014). and hemorrhage are commonly found. Extraskeletal
The tumor is more common in males, with a Ewing sarcoma shares the same cytogenetic marker
male:female ratio of 2:1 (Kransdorf and Meis 1993; [translocation of chromosomes t(11;22)(q24;q12)]
Kapoor etal. 2014). Histologically, the tumor com- with other Ewing sarcoma tumors (i.e., osseous
posed of chondroblast-like cells in an abundant Ewing sarcoma, soft tissue primitive neuroectoder-
myxoid matrix resembling embryonic cartilage. mal tumor, and Askin tumor).
However, despite its name, the WHO has classified Extraskeletal Ewing sarcoma commonly dem-
it as a tumor of uncertain differentiation due to its onstrates a nonspecific radiologic appearance of a
lack of cartilaginous differentiation. Additionally, large soft tissue mass affecting the paraspinal
cytogenetic studies have shown that the tumor has a region or lower extremity. CT commonly exhibits
reciprocal translocation, t(9;22)(q22;q12), not isoattenuation to the muscle but may demonstrate
observed in conventional skeletal chondrosarcoma low attenuation, likely corresponding to areas of
(Kapoor etal. 2014; Drilon etal. 2008). hemorrhage or necrosis. Calcification is atypical,
Radiography or CT shows a soft tissue mass occurring in approximately 10% of tumors at pre-
that does not contain calcifications or bone forma- sentation (Javery etal. 2011). MR imaging dem-
tion. The tumor appears on CT and MR imaging as onstrates heterogeneous signal intensity similar
a well-defined, large, and multilobular soft tissue to that of the muscle on T1-weighted images
mass. Highly myxoid lesions exhibit low attenua- and intermediate to high signal intensity on
T2-weighted images. The high cellularity of the within a hyalinized to chondromyxoid stroma.
tumor likely accounts for the intermediate signal The tumor resembles pleomorphic adenoma, and
intensity on T2-weighted image. Areas of hemor- a lack of obvious ductal differentiation leads to a
rhage appear as high signal intensity, and focal designation of myoepithelioma. Parachordoma
areas of necrosis are observed as low signal inten- closely resembles myoepithelioma and is best
sity on T1-weighted images and high signal inten- considered within this spectrum (Fisher and
sity on T2-weighted images. The presence of Miettinen 1997). This tumor is histologically
high-flow vascular channels is an additional image similar to chordoma but is observed in non-axial
feature of extraskeletal Ewing sarcoma. These soft tissues, usually occurring in the deep soft tis-
channels have low signal intensity for all pulse sues of the extremities and occasionally in the
sequences. This finding can be observed in hyper- retroperitoneum. Previous reports have revealed
vascular lesions, but the possibility of extraskeletal a male predilection, occurring in the fourth to
Ewing sarcoma should be considered if flow voids sixth decade of life. These tumors have a nonspe-
are observed in young adults with a large intra- cific imaging appearance on CT and MR imaging
muscular mass (Murphey etal. 2013). (Abbes etal. 2008; Clabeaux etal. 2008; Ali
etal. 2013).
13.9 Extrarenal Malignant

Rhabdoid Tumor 13.11 Pleomorphic Hyalinizing
Angiectatic Tumor
Extrarenal malignant rhabdoid tumors are extremely
rare. Most tumors occur in children less than 1 year The pleomorphic hyalinizing angiectatic tumor
of age and have a very poor prognosis (Oda and (PHAT) is a rare tumor of uncertain lineage
Tsuneyoshi 2006; Abdullah etal. 2010). The tumors described by Smith etal. in 1996 (Smith etal.
may arise at a variety of locations, including the 1996). The tumor is histologically composed of
liver, heart, and gastrointestinal system. Soft tissue clusters of ectatic, fibrin-lined, thin-walled
lesions seem to arise most frequently in deep, axial vessels surrounded by sheets and fascicles of

locations, including the trunk, extremities, neck, spindled and pleomorphic cells. A variable
and paraspinal regions (Kodet etal. 1991; Garces- inflammatory component is also observed (Smith
Inigo etal. 2009). Cutaneous lesions are also etal. 1996). The tumor occurs most commonly in
described. Clinically, the tumor presents as a rapidly the subcutaneous tissues of the lower extremities
growing soft tissue mass. in adults. The tumor may be associated with
CT images reveal a well-defined, large, and hemorrhage, giving the clinical impression of a
low-attenuated mass, and T2-weighted MR hematoma or Kaposis sarcoma. While the tumor
images show heterogeneous high signal intensity may be locally aggressive with a high rate of
(Garces-Inigo etal. 2009). The tumor is hetero- local recurrence (3350%), no case of metastatic
geneously enhanced after intravenous contrast disease has been reported (Folpe and Weiss 2004;
administration. The imaging characteristics of Subhawong etal. 2012).
soft tissue rhabdoid tumors have yet to be deter- The imaging findings of PHAT have not
mined, largely because of their rarity. been reported in detail in the radiology litera-
ture. A relatively small number of studies have
described nonspecific MR characteristics for
13.10 Myoepithelioma/ this tumor, namely, that it is isointense to the
Parachordoma muscle on T1-weighted images and heteroge-
neously hyperintense on T2-weighted images,
Tumors in this category are extremely rare tumors with enhancement on contrast-enhanced
composed of epithelial and myoepithelial ele- T1-weighted images (Subhawong etal. 2012;
ments in varying proportions; these tumors exist Suzuki etal. 2014).
13.12 Illustrations: Tumors ofUncertain Differentiation
13.12.1 Intramuscular Myxoma
a b
c d
Fig. 13.1Intramuscular myxoma. Axial T1WI (a) imal patchy enhancement (arrow) of the mass. US (d)
reveals the intramuscular mass to be homogeneously shows a low echoic mass with detectable internal echoes,
hypointense relative to the adjacent muscle. Axial FS a finding that is useful in differentiating it from the cystic
T2WI (b) shows a well-defined bright hyperintense soft mass. Surgical specimen (e) shows a well-circumscribed,
tissue mass located within the adductor magnus muscle. gelatinous mass with internal septa
Axial postcontrast FS T1WI (c) demonstrates typical min-
a b
c d
Fig. 13.2Intramuscular myxoma. Axial T1WI (a) magnus muscle. Coronal FS T2WI (c) reveals a brushlike
reveals the intramuscular mass to be homogeneously hyperintensity around the proximal and distal poles of the
hypointense relative to adjacent muscle. Axial and coro- lesion (arrows). This pattern is due to leakage of myxoid
nal T2WIs (b, c) show a well-defined fairly homogeneous tissue content. Axial postcontrast FS T1WI (d) shows
fluidlike hyperintense mass located within the adductor typical, moderate patchy enhancement of the mass
a b
c d
Fig. 13.3 Mazabraud syndrome. AP radiograph of the hypointense. Axial T2WI (c) shows multiple intramuscu-
pelvis (a) reveals multifocal osteolytic lesions with thin lar myxomas within the gluteus maximus and fibrous dys-
sclerotic margins involving the ilium and the right femur, plasia in the acetabulum (arrows). Axial postcontrast FS
in keeping with polyostotic fibrous dysplasia. On coronal T1WI (d) demonstrates variable enhancement of the
T1WI (b), the corresponding bone lesions appear to be tumors (arrowheads)
13.12.2 Synovial Sarcoma
a b
Fig. 13.4 Synovial sarcoma. Axial T2WI (a) reveals a a homogeneous, high signal intensity. Axial postcontrast
well-defined lobular soft tissue mass located in the subfas- FS T1WI (c) shows homogeneously intense
cial region that is iso- to hypointense to adjacent skeletal enhancement
muscle. Axial FS T2WI (b) shows the mass that exhibits
a b
c d
Fig. 13.5 Synovial sarcoma. Axial T1WI (a) shows a sity, arrowhead), hemorrhage or necrosis (high signal
large intramuscular mass in the thigh, with a focal area of intensity, star), and calcified or fibrotic collagenized
high signal intensity (arrow), suggestive of intratumoral regions (low signal intensity, arrows), which is often
hemorrhage. Axial and sagittal T2WIs (b, c) show a large observed in synovial sarcoma. Axial postcontrast FS
heterogeneous mass containing three different signal T1WI (d) demonstrates prominent heterogeneous
intensities. This triple sign is due to the combination of enhancement of the mass
areas of solid cellular elements (intermediate signal inten-
a b
c d
Fig. 13.6 Synovial sarcoma. Axial T1WI (a) shows geneity and a fluid-fluid level, creating a bowl of grapes
largely cystic areas with high signal intensity, suggestive appearance. Axial postcontrast FS T1WI (d) reveals large
of hemorrhagic components. Axial and sagittal T2WIs (b, non-enhancing hemorrhagic regions with fluid-fluid
c) show a multiseptated mass with marked signal hetero- levels
a b
c d
Fig. 13.7 Synovial sarcoma. Axial T1WI (a) shows a rhage or necrosis (high signal intensity), and calcified or
large mass in the right buttock, with a focal area of high fibrotic collagenized regions (low signal intensity) is seen.
signal intensity, suggestive of intratumoral hemorrhage Axial postcontrast FS T1WI (c) demonstrates both
(arrow). Axial T2WI (b) shows heterogeneous signal enhancing solid elements and non-enhancing hemor-
intensities known as a triple sign. A combination of solid rhagic or necrotic elements. Axial CT (d) shows a low-
cellular elements (intermediate signal intensity), hemor- attenuated mass with internal focal calcifications (arrows)
13.12.3 Phosphaturic Mesenchymal Tumor
a b
c d e
Fig. 13.8 Phosphaturic mesenchymal tumor/mixed con- diffuse osteopenia with remote fracture in the right proxi-
nective tissue tumor (PMTMCT). Lateral radiograph of mal femur (arrow). Axial T1WI (c) shows a lobulated
the ankle (a) shows a subtle soft tissue density in the pos- mass on the posterior aspect of distal fibula, with isointen-
terior ankle, with cortical saucerization (arrow) in the sity to the adjacent muscle. Axial FS T2WI (d) shows a
adjacent posterior cortex of the distal fibula. There are homogeneous hyperintense mass abutting the posterior
several transverse sclerotic lines in the distal tibia (arrow- cortex of the distal fibula. Axial postcontrast FS T1WI (e)
heads), indicating insufficiency fracture due to uncontrol- demonstrates homogeneous strong enhancement of the
lable osteomalacia. (b) AP radiograph of the pelvis shows mas
a b
d e
Fig. 13.9 Phosphaturic mesenchymal tumor/mixed con- medial cortex of the proximal femur (arrows) and lateral
nective tissue tumor (PMTMCT). Bone scan (a) shows border of scapula (arrowheads), indicating looser zone.
multiple hot uptakes in the ribs, proximal femora, and dis- Postcontrast FS T1WIs (d, e) show a well-circumscribed
tal tibiae, in keeping with insufficiency fracture due to enhancing soft tissue lesion (arrows) in the T5 left para-
osteomalacia. Radiographs (b, c) demonstrate symmetric spinal muscle, which was histologically confirmed as
transverse lucencies with sclerotic irregular margins in the PMTMCT
13.12.4 Epithelioid Sarcoma
a b
Fig. 13.10 Superficial epithelioid sarcoma. Axial T1WI hyperintense signal along the deep fascia of the right fore-
(a) shows a subcutaneous mass with equal signal intensity arm. Axial postcontrast FS T1WI (c) exhibits homoge-
to that of adjacent muscle. Axial FS T2WI (b) shows a neous contrast enhancement with mild infiltrative margin
superficial single nodular mass with a homogeneous
a b
c d e
Fig. 13.11 Deep-seated epithelial sarcoma. Axial T1WI fluid-fluid level (star) and a peritumoral edema-like signal
(a) shows a deep-seated infiltrative soft tissue mass (arrowheads). Axial and sagittal postcontrast FS T1WIs
involving the medial head of gastrocnemius muscle. An (d, e) reveal heterogeneous tumor enhancement with large
internal, increased signal intensity is seen, suggestive of hemorrhagic necrotic components (stars) and infiltrative,
hemorrhage (arrow). Axial and sagittal FS T2WIs (b, c) poorly defined margins (arrows)
show a mass containing hemorrhagic changes with a
a b
Fig. 13.12 Infiltrative epithelial sarcoma. Axial T1WI neurovascular bundle (arrow) and closely abuts the sciatic
(a) reveals a deep-seated soft tissue mass wrapping around nerve (arrowhead), with infiltrative margins. Axial post-
the medial thigh, showing a similar signal intensity to that contrast FS T1WI (c) demonstrates tumor enhancement
of the adjacent muscle. Axial T2WI (b) shows heteroge- with infiltrative, poorly defined margins extending to all
neous signal intensities. The tumor encases the femoral compartments of the thigh
13.12.5 Angiosarcoma
a b c
d e
Fig. 13.13 Alveolar soft part sarcoma. Axial T1WI (a) T1WI (d) reveals intense enhancement with multiple flow
reveals an intramuscular mass with a higher signal inten- voids around the mass, suggestive of prominent intra- and
sity compared with the adjacent muscles. Axial and coro- peritumoral vessels. Angiogram (e) demonstrates hyper-
nal FS T2WIs (b, c) show a large soft tissue mass with a vascularity with prominent feeding arteries (arrow), pro-
heterogeneous high signal intensity. Peritumoral feeding longed capillary staining (star), and a draining vein
arteries (arrows) and draining veins (arrowhead) can be (arrowhead)
observed on coronal FS T2WI (c). Axial postcontrast FS
a b c
d e
Fig. 13.14 Alveolar soft part sarcoma. Coronal T1WI observed. Sagittal postcontrast FS T1WI (d) demonstrates
(a) reveals an intramuscular mass in the soleus muscle, intense enhancement with multiple flow voids around the
with multiple intra- and peritumor flow voids (arrows). mass, suggestive of prominent intra- and peritumoral ves-
Sagittal and axial FS T2WIs (b, c) show a heteroge- sels (arrow). The metastatic masses also show intense
neously hyperintense tumor with multiple flow voids enhancement (arrowheads). Angiogram (e) demonstrates
(arrows). Metastatic masses (b) are also observed in the hypervascularity with prominent feeding arteries (arrow),
adjacent muscle and diaphysis of the tibia (arrowheads). draining veins (arrowhead), and prolonged capillary
Characteristic flow voids in and around the mass are staining (star)
13.12.6 Clear Cell Sarcoma

Fig. 13.15 Clear cell sarcoma. Axial T1WI
(a) reveals a soft tissue mass in the dorsal a
aspect of third inter-metacarpal space, which
abuts the third and fourth metacarpal bones,
adjacent to the extensor tendons. The mass is
slightly hyperintense to the adjacent muscle,
corresponding to the presence of intralesional
melanin. Axial T2WI (b) shows a mass with
low to intermediate signal intensity. Axial
postcontrast FS T1WI (c) demonstrates
intense and homogeneous enhancement
enveloping the third and fourth extensor
tendons (arrowheads)
b
c
Fig. 13.16 Clear cell

sarcoma. Axial T1WI a b
(a) reveals a soft tissue
mass in the sole. The
tumor has a slightly
higher signal intensity
compared with muscle
on T1WI.Axial and
coronal T2WIs (b, c)
show intermediate signal
intensity with internal
low signal foci. The
mass encases plantar
intrinsic ligament
(arrowheads in b) and
displaces the flexor
tendons. Coronal
postcontrast FS T1WI
(d) demonstrates intense
enhancement
c d
13.12.7 Extraskeletal Myxoid Chondrosarcoma
a b c
d e f
Fig. 13.17 Extraskeletal myxoid chondrosarcoma. Axial geneous but prominent enhancement (except a small hem-
T1WI (a) reveals a multilobulated intramuscular mass in orrhagic portion), indicating a highly myxoid tumor. DWI
the anterior thigh. The lesion shows hyperintense foci cor- (d) and ADC map (e) show nonrestricted diffusion due to
responding to hemorrhagic changes within the tumor the characteristic nature of the chondroid matrix, which
(arrow). Axial T2WI (b) shows a mass with high signal has a high fluid content. CT (f) reveals a well-defined and
intense lobules defined by thin fibrous septa of low signal low-attenuated mass without calcification or bone
intensity. Axial postcontrast FS T1WI (c) reveals hetero- formation
c d
Fig. 13.18 Extraskeletal myxoid chondrosarcoma. AP muscle. Sagittal T2WI (c) shows a high signal mass with
radiograph of the knee (a) shows nonspecific soft tissue thin fibrous septa of low signal intensity. There is a small
mass (arrow) in the medial knee that does not contain cal- area of necrotic change (star). Sagittal postcontrast FS
cifications or exhibits bone formation. Axial T1WI (b) T1WI (d) shows prominent enhancement with a periph-
reveals a subcutaneous mass in the medial aspect of the eral/septal enhancement pattern (arrowheads)
proximal tibia. The tumor is isointense to the adjacent
13.12.8 Extraskeletal Ewing Sarcoma
a b c
Fig. 13.19 Extraskeletal Ewing sarcoma. Coronal T1WI low signal intensity (arrowhead) on all pulse sequences,
(a) reveals a large intramuscular mass in the medial aspect suggestive of the presence of high-flow vascular channels,
of the proximal thigh. The mass shows heterogeneous sig- which is an additional image feature of extraskeletal
nal intensity that is similar to that of muscle, with small, Ewing sarcoma. Coronal postcontrast FS T1WI (c) shows
hyperintense hemorrhagic foci (arrows). Coronal T2WI prominent enhancement with non-enhancing hemorrhagic
(b) shows a mass with intermediate to high signal inten- changes
sity due to the high cellularity of the mass. The mass has
a b
Fig. 13.20 Extraskeletal Ewing sarcoma. Axial T1WI hypointense necrotic area. Axial postcontrast FS T1WI
(a) reveals an intramuscular mass in the calf. The mass is (c) shows prominent enhancement, except in the central
isointense to the adjacent muscle. Axial T2WI (b) shows necrotic area (arrow)
a heterogeneously hyperintense tumor with a central
13.12.9 Extrarenal Rhabdoid Tumor
a b c
d e f
Fig. 13.21 Extrarenal rhabdoid tumor. T1WIs (a, d) tumor. There is peritumoral edema in the adjacent muscle.
show an intramuscular mass in the medial upper arm. The Postcontrast FS T1WIs (c, f) demonstrate heterogeneous
signal intensity of the mass is equal to that of the adjacent enhancement, with a non-enhancing necrotic portion in
muscle. T2WIs (b, e) show a lobulated hyperintense mass the lower part of the mass (arrow)
with an ill-defined margin at the inferior portion of the
13.12.10 Myoepithelioma
a b
c d
Fig. 13.22 Malignant myoepithelioma. Sagittal T1WI intermediate to hyperintense to the adjacent muscle on
(a) and axial T2WI (b) reveal a deeply situated intermus- T2WI (b, c). The sciatic nerve is anterolaterally displaced
cular soft tissue mass adjacent to the sciatic nerve in the (arrow in b). Axial postcontrast FS T1WI (d) shows het-
posterior compartment of the right proximal thigh. The erogeneous enhancement
mass is heterogeneously isointense on T1WI (a) and
13.12.11 Pleomorphic Hyalinizing Angiectatic Tumor ofSoft Part
a b c
d e
Fig. 13.23 Pleomorphic hyalinizing angiectatic tumor of signal foci and a peripheral high signal rim. Axial and
soft part. Axial T1WI (a) shows a well-circumscribed sagittal postcontrast FS T1WIs (d, e) show heterogeneous
intramuscular mass within the biceps brachii muscle. The enhancement. The central portion of the mass is less
mass is heterogeneously isointense to the adjacent mus- enhanced, but the peripheral portion of the T2 hyperinten-
cle. Axial and sagittal T2WIs (b, c) show a heteroge- sity is well enhanced. Surgical specimen (f) reveals het-
neously hyperintense soft tissue mass with internal low erogeneous mass
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Undifferentiated/Unclassified
Sarcoma 14
Malignant fibrous histiocytoma (MFH), the 14.1 Undifferentiated

malignant counterpart of the so-called fibrohis- Pleomorphic Sarcoma
tiocytic tumors, was first described in 1963 and is
generally accepted as one of the most common Undifferentiated pleomorphic sarcoma (UPS)
types of soft tissue sarcoma in the extremities of was used synonymously with MFH, the out-
elderly patients (Weiss and Enzinger 1978; Gibbs dated terminology, in the past. UPS still accounts
etal. 2001). The concept and classification of for a significant proportion of sarcomas occur-
MFH have changed over the past five decades. ring in late adult life between the ages of 50 and
With recent advances in immunohistochemistry, 70 years. The tumor occurs most frequently in
cytogenetic, and molecular genetic studies, many the lower extremities, especially the thigh, fol-
tumors labeled MFH could be subclassified as lowed by the upper extremities. It usually mani-
lineage-specific sarcoma after reassessment and fests as a painless, slowly enlarging mass
close scrutiny. The World Health Organization (Goldblum 2014). The vast majority of UPS are
(WHO) significantly reorganized the nomencla- high-grade lesions, having a local recurrence rate
ture of MFH in 2002. The term MFH was that ranges from 19% to 31%, a metastatic rate
removed, and the lesions are now included in the from 31% to 35%, and a 5-year survival rate from
quite separate and new category of undifferenti- 65% to 70% (Le Doussal etal. 1996; Zagars etal.
ated/unclassified sarcomas. The myxoid variant 1996; Salo etal. 1999; Engellau etal. 2004).
of MFH was renamed myxofibrosarcoma and Microscopically, UPS manifests a highly vari-
moved into the fibroblastic/myofibroblastic able histologic appearance, although it shows fre-
tumor section. These lesions show no definable quent transitions from storiform to pleomorphic
line of differentiation using currently available areas (Matushansky etal. 2009; Goldblum 2014).
technologies. Dedifferentiated types of specific Therefore, UPS is a diagnosis of exclusion. The
sarcomas are not included in this category. Such diagnosis of UPS is made after exclusion of a spe-
undifferentiated tumors may have spindle cell, cific type of pleomorphic sarcoma (e.g., pleomor-
pleomorphic, round cell or epithelioid cytomor- phic leiomyosarcoma, pleomorphic liposarcoma),
phologies. A significant subset of radiation- dedifferentiated liposarcoma, and sarcomatoid
associated sarcomas is included in this category carcinoma.
(Rosenberg 2013; Fletcher 2014; Goldblum The imaging features of UPS are quite nonspe-
2014; Doyle 2014). cific. On CT imaging, the tumor appears as

254 14 Undifferentiated/Unclassified Sarcoma
a heterogeneous enhancing mass, with similar 14.2 U

ndifferentiated RoundCell
attenuation to the adjacent muscle. Depending on andSpindle Cell Sarcoma
the cellularity and presence of hemorrhage, necro-
sis, and/or calcification, the tumor shows low to These lesions show round cell or spindle cell
intermediate signal intensity on T1-weighted morphology but cannot be classified into any
images and heterogeneous high signal intensity on other category. The subclassification of round
T2-weighted images. The lesion usually exhibits cell and spindle cell sarcomas is important
contrast enhancement, which may be heteroge- because it can provide optimal treatment options
neous if areas of necrosis or hemorrhage are pres- and well-defined chemotherapeutic regimens in
ent. An infiltrative growth pattern or tail-like spread certain subsets of round cell (e.g., Ewing sar-
of the tumor along deep fascia is frequently observed coma/PNET, rhabdomyosarcoma) and spindle
in superficial UPS, a feature that is described as a cell sarcomas (e.g., synovial sarcoma). If tumors
tail-sign on MR imaging. Several studies describe appear to be undifferentiated by whatever tech-
the tail-sign as a tail-like, multidirectional, and niques are applied, they remain in this category.
extensive spread along the fascial plane on However, at least within the round cell category,
T2-weighted images. On contrast-enhanced new genetic markers are beginning to be recog-
T1-weighted images, a tapered fascial enhancement nized for a subset of undifferentiated round cell
more than 2mm thick is observed extending from (non-Ewing) sarcomas, specifically those that
the tumor margin. Recently, the tail-sign has harbor CIC-DUX4, BCOR-CCNB3, or BCOR-
become recognized as an important risk factor for MAML3 fusion genes. The identification of
postoperative positive margins and local recurrence these markers suggests that some undifferenti-
(Lefkowitz etal. 2013; Iwata etal. 2014; Yoo etal. ated/unclassified sarcomas will eventually be
2014; Imanishi etal. 2016). However, this sign is recognized as genetically distinct tumor types
not specific for UPS and is frequently observed in (Doyle 2014; Fletcher 2014; Specht etal. 2016;
myxofibrosarcoma or nodular fasciitis. Fletcher 2008).
14.3 Illustrations: Undifferentiated/Unclassified Sarcoma
14.3.1 Undifferentiated Pleomorphic Sarcoma
a b
c d
Fig. 14.1 Undifferentiated pleomorphic sarcoma. Axial appears to be heterogeneously enhanced on axial postcon-
T1WI (a) and T2WI (b) show an intramuscular mass with trast FS T1WI (c). There is no intratumoral mineralization
heterogeneous and mixed signal intensities. The mass on CT (d)
a b
c d
Fig. 14.2 Undifferentiated pleomorphic sarcoma. Axial like contrast enhancement spreading from the mass, indi-
T1WI (a) shows a nonspecific subcutaneous soft tissue cating a tail-sign (arrows). Three years after wide excision
mass. Axial and coronal T2WIs (b, c) demonstrate hyper- of the mass, localized periosteal thickening (arrows) was
intense mass. There is tapered fascial thickening along the found on follow-up MR image (eg), likely indicating a
crural fascia, extending from the subcutaneous mass local tumor recurrence. US (h) also shows localized peri-
(arrows). Axial postcontrast FS T1WI (d) demonstrates osteal soft tissue thickening (arrow), histologically con-
heterogeneous enhancement in the mass and reveals a tail- firmed as recurred tumor
e f
g
h
a b
c d
Fig. 14.3 Undifferentiated pleomorphic sarcoma. Axial d emonstrate a perifascial extension of signal abnormali-
T1WI (a) and T2WI (b) show a nonspecific subcutaneous ties spreading from the main mass, suggestive of the tail-
soft tissue mass in the posterior shoulder. The mass is sign (arrow). Sagittal postcontrast FS T1WI (d) also
isointense on T1WI (a) and heterogeneously hypo- to shows heterogeneous enhancement with central necrosis
hyperintense on T2WI (b). Sagittal FS T2WIs (c) and a tail-sign (arrow) along the adjacent deep fascia
a b
c d
Fig. 14.4 Undifferentiated pleomorphic sarcoma. Axial s ignal intensity. Axial postcontrast FS T1WI (c) reveals
T1WI (a) shows a large intramuscular mass of slightly heterogeneous enhancement. Non-contrast CT (d) shows
higher signal intensity than the surrounding muscle. Axial a nonspecific intramuscular mass without calcification
T2WI (b) shows heterogeneous intermediate to high
a b
c d
Fig. 14.5 Undifferentiated pleomorphic sarcoma. Axial (arrowheads). Axial (c) and coronal (d) postcontrast FS
T1WI (a) shows a poorly defined intramuscular mass T1WIs reveal diffuse contrast enhancement of the mass.
involving the adjacent femur cortex (arrow). Axial T2WI Direct bone invasion (arrow) is observed in the medial
(b) reveals an infiltrative soft tissue mass of intermediate cortex of the femur, with adjacent bone marrow
to high signal intensity encasing the neurovascular bundle enhancement
14.3.2 CIC-DUX4 Fusion Positive Undifferentiated RoundCell Sarcoma
a b
Fig. 14.6 CIC-DUX4 fusion positive undifferentiated Axial postcontrast FS TWI (c) shows central necrosis and
round cell sarcoma. Axial T1WI (a) and T2WI (b) show a peripheral enhancement
subcutaneous mass of heterogeneous signal intensity.
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Superficial Soft Tissue Masses
15
15.1 Epidermal Inclusion Cyst bulls eye appearance has been described for
the testis). Histologically, the floating linear
Epidermal inclusion cyst is a benign lesion echogenic reflections are created by sporadically
derived from the focal proliferation of dermal distributed cholesterol, sebaceous foci, or calcifi-
squamous epithelium. This tumor is also referred cations, while the dark clefts occur in areas con-
to as sebaceous, epidermoid, epidermal, infun- taining scattered fragments of packed lamellae of
dibular, or keratin cysts (Kim etal. 2011a). keratin (Lee etal. 2001; Huang etal. 2011). The
Epidermal inclusion cysts are lined with stratified internal debris is mobile, showing a swirling
squamous epithelium and filled with a white, appearance when pressed with the probe (Kim
cheesy material, reflecting layers of keratin and etal. 2011a). The cyst exhibits posterior acoustic
cholesterol-rich debris (Hong etal. 2006). The enhancement and a small extension into the der-
cyst may be a congenital lesion developed from mis, corresponding to its communication with
the subcutaneous implantation of keratinizing the skin (i.e., dermal attachment) (Lee etal.
epithelial cells during embryogenesis. Epidermal 2001). Color Doppler study usually shows no
inclusion cysts can also be a result of downward vascular flow signal, but this can be observed in
growth of epithelial cells after occlusion of the ruptured cysts.
hair follicle and the growth of implanted epithe- On MR imaging, the lesion is a well-
lial elements after trauma or surgery (Hong etal. demarcated oval-shaped mass of high signal
2006; Huang etal. 2011; Kim etal. 2011a). intensity on T2-weighted image. Moderate linear
Clinically, epidermal inclusion cysts present as dark debris can be observed in the nondependent
slow-growing dermal or subcutaneous cysts. portion of the mass and can be best visualized on
These cysts usually occur in the hair-bearing T2-weighted image. On T1-weighted image, the
areas of the body, such as the scalp, face, neck, internal signal intensity is either hyper- or isoin-
trunk, and back (Kim etal. 2011a). Moreover, tense to that of adjacent muscles due to its pro-
they usually remain asymptomatic unless they teinaceous content. After contrast administration,
become infected or rupture into the adjacent soft the mass shows thin peripheral enhancement.
tissues. However, ruptured epidermal cysts usually con-
On US, the cyst is a well-circumscribed, tain septa and show thick and irregular rim
ovoid, and hypoechoic mass containing variable enhancement, with fuzzy adjacent soft tissue
echogenic foci. Associated internal debris enhancement on postcontrast images. These
includes internal linear echogenic reflections, imaging features of a ruptured epidermal cyst
filiform anechoic areas, or alternating layers of could mimic a mass of infectious or neoplastic
hyper- and hypoechoic rings (an onion ring or origin. (Hong etal. 2006).

264 15 Superficial Soft Tissue Masses
15.2 Pilomatricoma image, which may be related to the large amount

of collagen within the lesion (De Beuckeleer etal.
Pilomatricoma is a benign superficial tumor of the 1996). Pilomatricoma has an internal reticulation
hair follicle. It frequently occurs in the dermal or and patchy areas on T2-weighted image and post-
subcutaneous layer of the neck, head, and face contrast T1-weighted image, which corresponded
(including the periorbital, preauricular, cheek, to the distribution of the intercellular stroma on
and scalp areas), followed by the upper extremity. pathologic specimens (Lim etal. 2007). Peritu
However, pilomatricoma can occur in any hair- moral hyperechogenicity on US and peritumoral
bearing skin, usually within the first two decades strands on CT and MR imaging can be found if
of life. Clinically, this tumor appears as an asymp- pilomatricoma is accompanied by chronic inflam-
tomatic palpable mass with a rock-hard consis- mation with foreign body reaction. The radiologic
tency. Histologically, the tumor is composed of differential diagnosis of pilomatricomas includes
epithelial cells surrounded by a connective tissue calcified lymph nodes, ossifying hematoma, hem-
capsule. There are basaloid cells in the peripheral angioma with phlebolith, granuloma annulare,
regions of the tumor, whereas the central region and dermatofibrosarcoma.
contains mummified ghost cells. These cells have
lost their nuclei and are filled with keratin and
often calcium deposits. The stroma is composed 15.3 Fat Necrosis
of collagen and contains blood vessels.
Calcification is observed in approximately 85% Fat necrosis infrequently presents as a soft tissue
of lesions (Hwang etal. 2005; Lim etal. 2007; mass and usually located over a pressure point or
Garioni etal. 2008; De Beuckeleer etal. 1996). bony protuberance. Clinically, the patient may be
Little is known about the ultrasonographic fea- asymptomatic or may present with pain, skin
tures of pilomatricoma despite its frequent occur- induration, ecchymosis, skin retraction, or skin
rence because this entity is usually excised thickening. Numerous etiologies of fat necrosis
without imaging studies. On US, pilomatricoma have been reported, including trauma, cold expo-
usually appears to be an oval hyperechoic or sure, iatrogenic injections, collagen vascular dis-
isoechoic mass with well-defined margin. Most ease, sickle cell disease, and vasculitis. The end
cases have internal echogenic foci that represent result has been described as vascular impairment
calcifications and posterior acoustic shadowing or saponification of fat by lipase originating from
behind these calcifications. Occasionally, it pres- blood (Walsh etal. 2008). Fat necrosis can be a
ents as a calcification only or as an irregular finding in lobular panniculitis, which is an inflam-
hyperechoic lesion with an obscured margin due mation in the fat lobules of subcutaneous fat
to posterior acoustic shadowing. A hypoechoic (Fernando etal. 2003). Histologic examination
rim is also frequently found, representing a con- reveals nonviable adipose tissue with chronic and
nective tissue capsule that surrounds the tumor focal inflammation, frequently surrounded by a
histopathologically. Color Doppler flows are reactive fibrous capsule (Chan etal. 2003;
commonly found in the peripheral region only Fernando etal. 2003; Walsh etal. 2008). It has
(Hwang etal. 2005). On CT, pilomatricoma is a variable appearances that range from nonspecific
well-defined mass of soft tissue density showing soft tissue stranding to mimicking lipoma or
varying levels of calcification and variable liposarcoma.
enhancement (Lim etal. 2007). Lim etal. reported The radiologic appearance of fat necrosis in
findings of cystic degeneration and peritumoral the extremities has not been well described, while
changes. On MR imaging, pilomatricoma shows a its sonographic and mammographic appearances
homogeneous intermediate signal intensity on in the breast are better documented (Fernando
T1-weighted image and heterogeneous low to etal. 2003). Fernando etal. and Walsh etal.
intermediate signal intensity on T2-weighted reported fat necrosis may have two different
15.5 Morel-Lavallee Lesion 265
sonographic appearances, either appearing as a central area of necrosis surrounded by chronic

poorly defined hyperechoic region within the inflammatory cells and palisading fibroblasts
more hypoechoic subcutaneous fat, or a well- (Sanders etal. 1998). US shows a moderately well-
defined isoechoic mass with a hypoechoic halo circumscribed, ovoid, hypoechoic, heterogeneous
(Fernando etal. 2003; Walsh etal. 2008). The lesion with a paucity of lesional vascularity on
signal characteristics of subcutaneous fat necro- color Doppler study. F-18 FDG PET/CT can show
sis on MR imaging are not clearly defined. Lopez moderate uptake (SUV max 4.2) mimicking malig-
etal. described fat necrosis in the extremities as a nant soft tissue tumor (Strobel etal. 2009).
small, linear spiculated lesion with either a glob-
ular T1 hyperintense area (corresponding to fat
necrosis) or a T2 hypointense laminar component 15.5 Morel-Lavallee Lesion
(representing reactive fibrous tissue) (Lpez etal.
1997). Tsai etal. described the MR features of Morel-Lavallee lesion is a traumatic lesion pat-
subcutaneous fat necrosis as a lack of a discrete tern that has been termed closed degloving
mass, linear hypointensity on T1-weighted injury because it is the result of abrupt separa-
images, and mixed hyper- or hypointensity on tion of the subcutaneous fatty tissue from the
T2-weighted images. The variability of linear underlying fascia (Parra etal. 1997; Mellado
signal intensities is most likely related to the vari-
etal. 2004; Neal etal. 2008). Disruption of the
able stages of necrosis, edema, hemorrhage, and rich lymphatic and vascular plexus occurs upon
fibrosis that are associated with fat necrosis afterseparation from the adjacent fascia; accumula-
trauma over time (Tsai etal. 1997). In cases of tion of blood and lymph can therefore be observed
poorly defined hyperechoic regions on US, Walsh (Parra etal. 1997). Long-standing lesions are
etal. described fat necrosis on MR imaging as an typically encapsulated with serosanguinous fluid
ill-defined mass with intermediate signal inten- containing blood and lymph (Mellado etal.
sity and either diffuse or ring enhancement after 2004). The lesion typically is observed in the
contrast administration (Walsh etal. 2008). proximal thigh and trochanteric region.
On MR imaging, the lesions show some
expansive effects, generating a palpable bulge or
15.4 Rheumatoid Nodule compressive deformity on the adjacent muscles.
The MR signal intensities of the lesions may be
Rheumatoid nodules are uncommon but well- variable depending on the composition and stage
known extra-articular manifestations of rheuma- of development (Mellado etal. 2004). Long-
toid arthritis, commonly occurring at sites of standing lesions may present fluid-like MR sig-
repetitive mechanical irritation, such as the nal characteristics with a hypointense peripheral
extensor surfaces of the hand, feet, elbow, and the rim, a finding that is correlated with a fibrous
ischial tuberosities (Veys and De Keyser 1993). capsule. Morel-Lavallee lesions may also show a
Radiographs show nonspecific soft tissue mass homogeneous high signal intensity on both T1-
that is uncommonly calcified. On MR imaging, and T2-weighted images, indicating the presence
rheumatoid nodule appears as an ill-defined solid of methemoglobin in a subacute hematoma. The
mass or a predominantly cystic mass. The solid third MR signal intensity pattern is heteroge-
mass shows a low signal intensity on T1-weighted neous hyperintensity on T2-weighted image,
image, low to intermediate signal intensity on which reflects hemosiderin deposition, granula-
T2-weighted image and is well enhanced after con- tion tissue, necrotic debris, fibrin, and blood
trast administration. These findings are correlated clots, which are characteristics of chronic orga-
with predominantly chronic inflammatory tissue nizing hematoma. The hypointense peripheral
observed on microscopic examination (El-Noueam ring can represent a hemosiderin-laden fibrous
etal. 1997). The predominantly cystic mass has a capsule with mild inflammatory infiltrate. Patchy
internal enhancement and peripheral enhance- nodules present as subcutaneous hypoechoic

ment may also be present (Mellado etal. 2004). nodules, often with low-level internal echoes due
US also shows a variable appearance, being more to the poor beam reflection of melanin (Catalano
homogeneous and flat or fusiform in shape, with etal. 2010a; Catalano and Siani 2010; Catalano
well-defined margins in older lesions. All Morel- etal. 2010b). Typical metastatic lymph nodes
Lavallee lesions are hypoechoic or anechoic, show a round or broad oval shape, with macrol-
compressible, and located between the deep fat obulated or microlobulated borders and a com-
and overlying fascia (Neal etal. 2008). plete or partial loss of the internal echoic hilum
(Catalano etal. 2010a; Catalano etal. 2010b).
Color Doppler US may be helpful in differentiat-
15.6 Malignant Melanoma ing the metastatic lymph node from reactive
lymph node hyperplasia. On MR imaging, mela-
Malignant melanoma is a malignant neoplasm of noma usually appears as a hyperintense lesion on
melanocytes, most frequently arising from the T1-weighted image, which is a characteristic sig-
skin as a growing pigmented lesion. However, it nal pattern produced by melanin. The tumor
can also appear pink, tan, or even white (http:// shows diffusion restriction on diffusion-weighted
www.cancer.org/cancer/skincancer-melanoma/ imaging (DWI) and intense enhancement after
detailedguide/melanoma-skin-cancer-what-is- contrast agent administration (Choi and
melanoma). Primary tumors most frequently Gershenwald 2007; Juan etal. 2014). PET/CT
present in Caucasian women on the leg, whereas represents the newest imaging modality used in
most melanomas most commonly occur on the the staging of melanoma, exhibiting between
trunk in men (Kalkman and Baxter 2004; 95% and 100% sensitivity, specificity, and accu-
Catalano etal. 2010a). Melanoma can be cured in racy for N- and M-staging (Veit-Haibach etal.
its early stages with a 99% survival rate, but it is 2009). PET can provide added value for detecting
likely to spread to other parts of the body if it is in-transit or satellite metastatic lesions (Veit-
not detected early (Kalkman and Baxter 2004; Haibach etal. 2009).
Catalano etal. 2010a). At initial diagnosis, cross-
sectional imaging modalities, such as CT, MR
imaging, PET, and PET/CT, may help assess 15.7 Lymphoma (Cutaneous
patients with cutaneous malignant melanomas andSubcutaneous)
for locoregional and distant metastases (Choi and
Gershenwald 2007; Veit-Haibach etal. 2009; Cutaneous T-cell lymphomas (CTCLs) repre-
Catalano etal. 2010a). sent a heterogeneous group of neoplasms that
On US, a primary tumor appears as a originate from skin-homing T cells (Willemze
hypoechoic lesion bordered by a thin and echoic and Meijer 2006). CTCL is the most common
epidermal surface. The thickness of the primary type of cutaneous lymphoma and typically pres-
lesion is an important parameter in management ents with dry skin, itching, a red rash, and thick
and prognosis of cutaneous melanoma (Choi and plaques that often mimic eczema or chronic der-
Gershenwald 2007; Veit-Haibach etal. 2009; matitis. The disease affects men more than
Catalano etal. 2010a; Catalano and Siani 2010). women and usually occurs in men in their 50s
US is also helpful in assessing locoregional and 60s. Subcutaneous T-cell lymphoma is also
metastasis. Satellite metastasis is defined when a known as a panniculitis-like T-cell lymphoma,
metastatic nodule is found 23cm from the pri- which infiltrates the subcutaneous fat but does
mary tumor. In-transit metastasis is reported if not involve the skin. This disease most com-
the lesion develops along the lymphatic course monly presents in young adults with subcutane-
toward the locoregional lymph node basin (Meier ous nodules, primarily located on the trunk,
etal. 2002; Catalano etal. 2010a). Metastatic extremities, and face (Willemze and Meijer
15.7 Lymphoma (Cutaneous andSubcutaneous) 267
Table 15.1 WHO-EORTC classification of cutaneous ated with connective tissue disease. Most cuta-
lymphomas with primary cutaneous manifestations
neous lymphomas typically fall into the category
Cutaneous T-cell and NK cell lymphomas of indolent lymphomas and respond well to
Mycosis fungoides mild treatments. In contrast, cutaneous diffuse
MF variants and subtypes large B-cell lymphomas are characterized by an
Folliculotropic MF aggressive clinical course and have a more unfa-
Pagetoid reticulosis
Granulomatous slack skin
vorable prognosis than other groups of cutane-
Szary syndrome ous lymphomas (Zinzani etal. 2006; Willemze
Adult T-cell leukemia/lymphoma 2006). Subcutaneous B-cell lymphomas have
Primary cutaneous CD 30+ lymphoproliferative rarely been reported and seem to be character-
disorders
Primary cutaneous anaplastic large cell lymphoma
ized by rapid growth (Alaibac etal. 2008).
Lymphomatoid papulosis On US, subcutaneous lymphomas show a
Subcutaneous panniculitis-like T-cell lymphoma poorly defined hyperechogenicity, with or without
Extranodal NK/T-cell lymphoma, nasal type central hypoechoic areas in the subcutaneous fat
Primary cutaneous aggressive epidermotropic
CD8-positive cytotoxic T-cell lymphoma (provisional
layer (Kang etal. 2007; Kim etal. 2011b). The
entity) hyperechogenicity of the tumors reflects the differ-
Cutaneous g/d T-cell lymphoma (provisional entity) ence in acoustic impedance between adipose cells
Primary cutaneous CD4-positive small/medium-sized and lymphocyte clusters. The central hypoechoic
pleomorphic T-cell lymphoma (provisional entity) areas result from the decrease in acoustic imped-
Primary cutaneous peripheral T-cell lymphoma, ance due to the dense accumulation of lympho-
unspecified
cytes, and lymphocytoid cells in the central portion
Cutaneous B-cell lymphoma
of the mass reflect its hypoechoic appearance
Primary cutaneous marginal zone B-cell lymphoma (Fujii etal. 2004). Color Doppler US demonstrates
Primary cutaneous follicle center lymphoma relative hypervascularity with a low resistive
Primary cutaneous diffuse large B-cell lymphoma, leg
type index. (Kang etal. 2007). On CT, lymphomas
Primary cutaneous diffuse large B-cell lymphoma, other appear as enhancing nodules and infiltrative cuta-
Anaplastic or plasmablastic subtypes neous and subcutaneous masses. The tumors show
T-cell/histiocyte rich large B-cell lymphoma nonspecific imaging features on MR imaging,
Intravascular large B-cell lymphoma
appearing as a discrete mass or thickening of the
skin and subcutaneous tissue, with T1 hypo- and
2006; Kim etal. 2011b; Jang etal. 2012; Juan T2 hyper-intense signal intensities. Enhancement
etal. 2014). Cutaneous B-cell lymphomas is observed after contrast agent administration
(CBCLs) are the less common version of cuta- (Juan etal. 2014). PET/CT is more accurate than
neous lymphomas, constituting approximately CT in the staging and assessment of the treatment
2025% of all primary cutaneous lymphomas. response of cutaneous lymphoma because these
The disease presents in the skin, without evi- tumors are typically FDG avid. Measuring the
dence of extracutaneous disease at the time of maximum SUV can be a useful parameter to assess
diagnosis (Willemze and Meijer 2006). The the aggressiveness of the lymphoma. (Juan etal.
detection of extracutaneous involvement by 2014; Kim etal. 2011b).
imaging is crucial for staging and therapeutic However, there are no specific imaging fea-
planning for lymphoma (Juan etal. 2014). The tures of cutaneous/subcutaneous lymphomas. For
World Health Organization (WHO)-European this reason, the differential diagnoses should
Organization for Research and Treatment of include panniculitis, subcutaneous edema, hem-
Cancer (EORTC) classification of cutaneous orrhage, cellulitis, and lipoma (Kang etal. 2007).
lymphomas is summarized in Table15.1. Moreover, the clinical findings are important, and
Clinically, subcutaneous lymphomas are often the biopsy must be recommended with a high
confused with inflammatory panniculitis associ- index of clinical suspicion.
15.8 Soft Tissue Metastasis source of cutaneous metastasis in men, with

breast carcinoma being the most common source
Soft tissue metastasis is a relatively uncommon in women (Alcaraz etal. 2012). However, gas-
cause of soft tissue masses. The primary tumor of tric, hepatic, and pancreatic cancers have a lower
origin of soft tissue metastasis is the skin, lung, probability of spreading into the skin (Kovcs
and breast, followed by the kidney, colon, and etal. 2013). Common sites of cutaneous metas-
rectum. However, metastases may spread from tases are variable depending on their primary
any primary tumor location (Beaman etal. 2007). tumor type and locations. Generally, lower gas-
Sarcomas are an uncommon cause of metastases trointestinal and genitourinary cancers prefer
to the soft tissues. Soft tissue metastasis may be infradiaphragmatic skin regions, unlike upper
misidentified as primary soft tissue sarcomas or gastrointestinal cancers, whereas lung and kid-
inflammatory lesions based on the physical find- ney cancers prefer supradiaphragmatic regions.
ings and imaging studies. A multiplicity of Cutaneous metastases generally arise in a late
lesions and known primary tumors may help in stage of cancer with a poor prognosis but may
diagnosing soft tissue metastases. also be the first clinical manifestation of an
Metastases to the skeletal muscle have been asymptomatically progressing cancer (Kovcs
generally considered rare, but recent investiga- etal. 2013).
tions show that they are more common than pre- On CT, soft tissue metastasis generally
viously suggested (Haygood etal. 2012). These appears as an area of decreased attenuation. On
metastases usually occur in the abdominal wall, MR imaging, soft tissue metastases often present
back/ periscapular region, thigh, and chest wall. as a nonspecific soft tissue mass. In some cases,
Metastases distal to the knee and elbow are they appear as abscess-like lesions with rim
extremely rare (Gottlieb and Schermer 1970). enhancement (Surov etal. 2010). They may pres-
Metastasis to the skin occurs in 0.79% of all ent with multiple intramuscular calcifications,
patients with cancer (Damron and Heiner 2000; which have been described sporadically in gastric
Beaman etal. 2007; Plaza etal. 2008). There are cancer, esophageal, and colonic adenocarcinoma
several mechanisms of cutaneous metastasis, (Surov etal. 2010). In metastases of mucinous
including distant organ metastasis, local inva- adenocarcinoma of the colon or pancreas, marked
sion, locoregional lymphatic dissemination, and hypointensity is observed on T2-weighted image
local iatrogenic cancer cell contamination in the as a consequence of the high ratio between nuclei
surgical scar (Kovcs etal. 2013). If melanoma and cytoplasm and limited extracellular space
is excluded, lung cancer is the most frequent (Damron and Heiner 2000).
15.9Illustrations: Superficial Soft Tissue Masses 269
15.9 Illustrations: Superficial Soft Tissue Masses
15.9.1 Epidermal Inclusion Cyst
a b
c d
Fig. 15.1 Epidermal inclusion cyst. Axial T1WI (a) (c) demonstrates only thin peripheral rim enhancement.
shows a subcutaneous mass in the medial knee, with US (d) shows a heterogeneously hyperechoic mass with
slightly higher signal intensity compared to the muscle. scattered internal linear echogenic reflections (arrow),
Axial T2WI (b) shows a hyperintense mass with internal dark clefts (arrowhead), and increased posterior acoustic
hypointense debris (arrows). Axial postcontrast FS T1WI enhancement
a b
c d
Fig. 15.2 Epidermal inclusion cyst. Axial T1WI (a) rim. Mild peri-lesional edema is observed. The mass
shows a subcutaneous, hyperintense mass in the plantar shows irregular rim enhancement with fuzzy adjacent
aspect of first toe. Axial and coronal T2WIs (b, c) reveal a soft-tissue enhancement on axial postcontrast FS T1WI
hyperintense mass with a peripheral hypointense fibrotic (d)
a b
c d
Fig. 15.3Ruptured epidermal inclusion cyst. Axial tissue enhancement on axial postcontrast FS T1WI (b).
T2WI (a) shows a subcutaneous mass with irregular con- US images (c, d) also shows irregular margins and peri-
tours and extensive peri-lesional edema in the left poste- lesional hypervascularity, indicating a ruptured epidermal
rior shoulder. The mass contains septa and shows thick cyst. These imaging features of a ruptured epidermal cyst
and irregular rim enhancement with fuzzy adjacent soft- could mimic a mass of infectious or neoplastic origin
15.9.2 Pilomatricoma
a b
Fig. 15.4 Pilomatricoma. Axial T1WI (a) and T2WI (b) shows peripheral thick rim enhancement with central
show a small, well-demarcated, subcutaneous mass with non-enhancement
low signal intensity. Axial postcontrast FS T1WI (c)
a b
c d
e f
Fig. 15.5 Pilomatricoma. Variable sonographic appear- in the superficial subcutaneous tissue (ce). A hypoechoic
ances of pilomatricoma. Completely calcified pilomatri- rim (arrows) is also frequently found, which represents a
coma with posterior acoustic shadowing artifact (a). connective tissue capsule surrounding the tumor. (f) The
Irregular hyperechoic lesion with an obscured margin due surgical specimen reveals a nodulocystic tan-colored
to posterior acoustic shadowing (b). Well-defined iso- to mass containing irregularly shaped, lobulated islands of
hyperechoic masses with variable internal echogenic foci cells surrounded by a fibrous capsule
a b
c d
Fig. 15.6 Pilomatricoma. Cystic variant of pilomatri- contrast FS T1WI (c) shows thin peripheral rim
coma is observed on MR imaging. Axial T1WI (a) and enhancement. US (d) shows fluid-containing cyst with a
T2WI (b) reveal a solid-cystic mass with a hypointense hyperechoic peripheral solid portion surrounded by a
nodular portion (arrowheads) associated with a T2 hyper- hypoechoic rim (arrows). The surgical specimen (e)
intense fluid-filled cystic component (star). Axial post- shows a well-demarcated solid mass with a central cleft
15.9.3 Fat Necrosis
a a
b
b
Fig. 15.7 Fat necrosis. Axial contrast-enhanced CT (a)

scan of an abdomen shows a hypodense lesion in the sub-
cutaneous tissue. Rim enhancement (arrow) and perile-
sional stranding can be seen. US (b) shows a hyperechoic,
c
heterogeneous poorly defined region
Fig. 15.8 Fat necrosis. Axial T1WI (a) and T2WI (b)
show a left chest wall lesion with low signal intensity and
infiltrative margin (arrows). Axial postcontrast FS T1WI
(c) shows diffuse enhancement of the mass
15.9.4 Rheumatoid Nodule
a b
c d
Fig. 15.9 Rheumatoid nodule. AP oblique radiograph of T1WI (b) and low to intermediate signal intensity on
the foot (a) shows a bulging soft tissue density over the T2WI (c). Axial postcontrast FS T1WI (d) reveals rim
peroneal tuberosity in the lateral foot. Axial T1WI (b) enhancement surrounding the non-enhancing central
reveals a subcutaneous soft tissue mass with bulging con- necrotic tissue
tours. The solid mass shows a low signal intensity on
a b
Fig. 15.10 Rheumatoid nodule. Transverse (a) and longitudinal (b) US images reveal a heterogeneous, predominantly
hypoechoic mass over the extensor surface of the elbow. Color Doppler study (c) shows limited peripheral vascularity
15.9.5 Morel-Lavallee Lesion
a b
c d
Fig. 15.11Morel-Lavallee lesion. Axial T1WI (a) signal rim. Coronal T1WI (c) shows that the mass abuts
shows a large lobulated cystic mass of high signal inten- the deep fascia with inferolateral extension, resulting in a
sity in the lateral aspect of the lateral thigh. Axial T2WI bulging mass. Axial postcontrast FS T1WI (d) shows no
(b) shows markedly increased signal intensity with a dark enhancement, except thin peripheral rim enhancement
15.9.6 Malignant Melanoma
a b
Fig. 15.12 Malignant melanoma. Clinical photograph (a) shows a dark black, asymmetric, and irregularly bordered
skin lesion in the heel. US images (b, c) shows hypoechoic skin mass with marked vascularity
a b
Fig. 15.13 Malignant melanoma. On MR imaging, a and FS T2WI (b), which is a characteristic of signal inten-
fungating skin lesion (arrows) is observed on the lateral sity produced by melanin, and is mildly enhanced after
aspect of the sole at the level of the calcaneocuboidal gadolinium administration (c)
joint. The mass exhibits hyperintensity on both T1WI (a)
a b
c d
Fig. 15.14 Malignant melanoma. There is soft tissue T2WI (a, b). The mass is mildly enhanced on postcontrast
thickening in the fourth toe. The mass shows mild hyper- FS T1WI (c). Surgical specimen also shows soft tissue
intensity compared to adjacent muscle on both T1WI and thickening in the distal phalanx of the fourth toe (d)
Fig. 15.15In-transit
metastasis of malignant a
melanoma. US (a) of
patient with a history of
malignant melanoma
reveals a subcutaneous
hypoechoic nodule
along the dilated
lymphatic course
(arrow) toward the
locoregional lymph node
basin, consistent with
in-transit metastasis. The
nodule shows marked
hypervascularity on
color Doppler study (b)
b
15.9.7 Lymphoma (Cutaneous andSubcutaneous)
a b
c d
Fig. 15.16 Peripheral T-cell lymphoma. Axial T1WI (a) T1WIs show diffuse and intense enhancement. Color
and T2WI (b) show ill-defined subcutaneous soft tissue Doppler study (e) reveals hyperechoic mass with branch-
mass and perilesional fat stranding in the anterior aspect ing hypoechoic structures in the subcutaneous fat layer
of the elbow. Axial (c) and sagittal (d) postcontrast FS and relative hypervascularity
a b
c d
Fig. 15.17 Diffuse large B-cell lymphoma. Axial T1WI to adjacent muscle. Axial (c) and sagittal (d) postcontrast
(a) and FS T2WI (b) show a large tumor involving the FS T1WIs reveal that the mass has diffused and intense
ulnar side of the right upper arm. The tumor involves the enhancement. Color Doppler study (e) reveals heteroge-
skin and subcutaneous tissue with hypointensity on axial neous echoic mass with branching hypoechoic structures
T1WI (a). On axial FS T2WI (b), the mass is hyperintense in the subcutaneous layer and relative hypervascularity
b c
Fig. 15.18 Panniculitis-like T-cell lymphoma. PET-CT a poorly defined hyperechogenicity with multiple, linear
image (a) shows two subcutaneous masses with intense hypoechoic areas in the central portion. US obtained in
FDG uptake in the abdominal wall. The corresponding the left lateral buttock (c) shows ill-defined hyperechoic
US obtained in the left anterior abdominal wall (b) reveals infiltration in the subcutaneous fat layer
a b
c d
Fig. 15.19 Diffuse large B-cell lymphoma. The patient US (c) reveals diffuse thickening of chest wall muscles
presents with a rapidly growing chest wall mass and skin and intervening hypoechoic linear structures, indicating
color change (a). Contrast-enhanced chest CT (b) shows preservation of muscle fascicular structures. Color
diffuse soft tissue and skin thickening. The corresponding Doppler study (d) reveals hypervascularity of the mass
15.9.8 Soft Tissue Metastasis
a b
c d
Fig. 15.20 Intramuscular metastasis of rhabdomyosar- reveals hyperintense mass with a small central cystic por-
coma. Axial T1WI (a) shows a nonspecific soft tissue tion. Axial postcontrast FS T1WI (c) demonstrates thick
mass in the flexor carpi ulnaris muscle. The mass is isoin- peripheral enhancement. Prominent vascularity is found
tense to the adjacent skeletal muscle. Axial FS T2WI (b) on color Doppler study (d)
References 287
a b
c d
Fig. 15.21 Subcutaneous metastasis of malignant phyl- cific signal intensity. Axial postcontrast FS T1WI (c)
lodes tumor from the breast. Axial T1WI (a) shows a non- shows diffuse and intense contrast enhancement. Surgical
specific soft tissue mass in the subcutaneous layer of the specimen (d) shows multilobulated solid mass
left upper arm. Axial T1WI and T2WI (b) reveal nonspe-
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Masses That May Mimic Soft Tissue
Tumors 16
16.1 Ganglion On US, ganglion is usually an anechoic mass

with posterior acoustic enhancement, but small
Ganglion is a benign cystic mass containing a cysts (less than 10mm in the largest dimension)
gelatinous fluid rich in hyaluronic acid and other may have echogenic foci without posterior acous-
mucopolysaccharides, with a fibrous capsule tic enhancement. Larger ganglion cysts are more
without synovial lining (McCarthy and McNally likely to have well-defined borders and multiple
2004). The mass arises from the joint capsule, lobulations (Wang etal. 2007).
ligaments, tendon sheaths, bursae, or subchon- On MR imaging, ganglion typically presents
dral bone (Bermejo etal. 2013). Ganglion is usu- as well-defined rounded or lobulated fluid collec-
ally found in the periarticular soft tissues in areas tions with sharply defined internal septations
under repetitive stress, with or without communi- (bunch of grapes appearance) (Perdikakis and
cation with joint spaces. Occasionally, they can Skiadas 2013; McCarthy and McNally 2004).
occur far from a joint or even in an intramuscular These cysts show a homogeneous high signal
location. The origin of the fluid has been postu- intensity on T2-weighted image but may have a
lated to arise from one of following three mecha- more heterogeneous appearance. On T1-weighted
nisms. First, it may be pumped into the cyst from image, the signal intensity of ganglion depends
the joint by motion of the wrist. Second, it may on its protein concentration and the presence of
result from an extra-articular degenerative pro- hemorrhagic contents. Complication, including
cess, cyst formation, and subsequent communi- bleeding or inflammatory changes may also be
cation with the joint. Lastly, it may be the result present (Bermejo etal. 2013). Complicated gan-
of excessive mucin production by mesenchymal glion may have a more heterogeneous appear-
cells within the wall (Gude and Morelli 2008). ance, with a thick peripheral rim or internal septa
Intraneural ganglion cysts are rarely found. The in cases of infectious or inflammatory changes.
common peroneal nerve is most frequently However, they should not demonstrate internal
involved, and other affected nerves are often enhancement after contrast administration
located near articular spaces. The cystic lesion is (Bermejo etal. 2013).
located within the epineurium, causing an eccen-
tric displacement of the nerve fascicles. The pro-
posed pathogenesis of intraneural ganglion cyst 16.2 Vascular Lesion
is a dissection of synovial joint fluid into the epi-
neurium of an articular branch nerve supplying a Aneurysms or pseudoaneurysms may be erro-
joint, with subsequent centripetal dissection into neously diagnosed as a soft tissue tumor in the
the parent nerve (Spinner etal. 2007a, b). extremities. True aneurysms contain all three

292 16 Masses That May Mimic Soft Tissue Tumors
layers of the arterial wall (intima, media, and 16.3 Gout

adventitia). In contrast, pseudoaneurysms con-
tain only adventitia and is considered to be a Gout is characterized by the inflammatory
hematoma between the arterial wall and sur- response that results from the deposition of
rounding parenchyma, lined by inflammatory monosodium urate crystals in soft tissues and
cells and fibroblasts, analogous to an intramural joints (Desai etal. 2011). Tophaceous gout repre-
hematoma (Kransdorf and Murphey 2014; sents the chronic phase of the disease process.
Anderson etal. 2003). The popliteal artery is Pathologically, gouty tophi are aggregations of
the most frequently affected by peripheral urate crystals and a proteinaceous matrix, sur-
aneurysm. Patients are generally older men in rounded by an intense inflammatory reaction.
the fifth or sixth decades. Pseudoaneurysms The development of a tophus in the absence of
may be associated with a history of a single prior episodes of gouty arthritis is unusual (Yu
direct trauma or, more commonly, chronic blunt etal. 1997). Rarely, a soft tissue tophus may
trauma (Anderson etal. 2003). The lesions may occur in the absence of articular disease, which
remain stable for years, and symptoms may may result in a misdiagnosis of infection or neo-
relate to the overall size of the lesion. Clinical plasm (Yu etal. 1997). Gouty tophus may be
clues to the diagnosis include pulsatility and observed around joints and have a predilection
bruit in close proximity to an artery in a patient for the olecranon and prepatellar regions.
with a history of trauma. In these cases, it is Radiographs of gouty tophus usually demon-
important to make the diagnosis prospectively strate a juxta-articular soft tissue mass.
and to avoid biopsy (Wu and Hochman 2009). Calcification of the mass may be observed but is
The hypothenar hammer syndrome is a rare uncommon. On T1-weighted image, a tophus
clinical entity associated with thrombosed shows an intermediate signal intensity. The sig-
pseudoaneurysm in the ulnar artery. This syn- nal intensity on T2-weighted image is variable,
drome describes signs and symptoms associ- from low to high signal intensity, depending on
ated with ischemia of the hand and fingers the extent and distribution of calcification,
resulting from blunt repetitive injury of the whether the calcification is hydrated, and whether
ulnar artery and superficial volar arch against there is associated edema or inflammatory reac-
the hook of hamate (Anderson etal. 2003). tion (Yu etal. 1997). However, a heterogeneous
Aneurysms or pseudoaneurysms can be rec- intermediate to low signal intensity is the most
ognized on MR imaging with characteristic common pattern on T2-weighted image, result-
imaging features. Aneurysms present as well- ing from the presence of urate crystals and fibrous
defined round to elliptical masses with a persis- tissue (Narvez etal. 2003). After gadolinium
tent flow signal void. On T1- and T2-weighted administration, intense enhancement may be
images, they have a complex signal intensity seen, reflecting the surrounding granulation tis-
caused by blood products of varying ages: sig- sue and increased vascularity of the affected
nal void in regions of flowing blood, hyperin- synovium (Narvez etal. 2003). Recently, dual-
tensity on T1- and T2-weighted images due to energy CT has been introduced to differentiate
subacute hemorrhage, and hypointensity caused urate crystals from calcium using specific attenu-
by hemosiderin deposits (Stacy and Kapur ation characteristics. Dual-energy CT may be
2011). Moreover, MR images reveal a charac- used in patients with an unclear diagnosis or for
teristic flow-related artifact in the direction of excluding gout and a volumetric analysis of tophi
the phase encoding gradient (Sundaram and in patients with confirmed gout. Dual-energy CT
Sharafuddin 1995). The imaging distinction promises to be a unique and clinically relevant
between an aneurysm with extensive thrombus modality in the diagnosis and management of
and a pseudoaneurysm may be difficult. gout (Desai etal. 2011).
16.5 Mortons Neuroma 293
16.4 Sarcoidosis Cutaneous involvement is observed in 25% of

cases, typically at the time of diagnosis (Newman
Sarcoidosis is a systemic granulomatous disorder etal. 1997; Ahmed and Harshad 2006). Among
which can affect multiple organs (Moore and these cases, subcutaneous sarcoidosis is the least
Teirstein 2003; Koyama etal. 2004). common subset of cutaneous lesions, with a 1.4
Musculoskeletal involvement in sarcoidosis usu- 6% of incidence in patients with systemic sar-
ally occurs in patients with generalized disease coidosis (Chen etal. 2009). Subcutaneous
(Koyama etal. 2004). involvement is diagnosed by identifying nonin-
In skeletal muscles, sarcoidosis can occur in fectious sarcoidal or epithelioid granulomas with
the following two different forms: myopathic or minimal lymphocytic inflammation that predom-
nodular type involvement. The nodular type often inantly involves the panniculus. Ahmed and
causes solitary or multiple nodules; therefore, the Harstad reported 21 cases of subcutaneous sar-
most common clinical presentation is a palpable coidosis (Ahmed and Harshad 2006). These
mass without pain or with mild tenderness. The authors reported that subcutaneous sarcoidosis is
myopathic type involves muscles symmetrically characterized by a peak incidence during the
and diffusely and does not form a mass. Slowly fourth decade, female predisposition, slightly
progressive myalgia, weakness, and atrophy are tender subcutaneous lesions that typically involve
usually observed (Otake 1994; Moore and the upper extremities, cutaneous lesional multi-
Teirstein 2003). Nodular type muscular sarcoid- plicity and clustering, a strong association with a
osis has a characteristic appearance at MR imag- systemic disease component at the outset of dis-
ing that may allow accurate diagnosis (Koyama ease, and, notably, bilateral hilar adenopathy
etal. 2004). On axial T2-weighted image, the (Ahmed and Harshad 2006). On MR imaging,
nodule has a star-shaped central region of low sig- sarcoidal subcutaneous granulomatous infiltra-
nal intensity, surrounded by a bright rim (i.e., tion, skin nodules, and soft tissue masses associ-
dark star sign). Only the peripheral hyperintense ated with lymphadenopathy may be detected.
rim shows intense enhancement after gadolinium These lesions show increased signal intensity on
administration. Histologically, a star-shaped T2-weighted image, low signal intensity on
hypointense center corresponds to fibrous tissue, T1-weighted image, and enhancement after gad-
and the peripheral hyperintense rim is believed to olinium administration (Moore and Teirstein
reflect active inflammatory granulomas and 2003). US demonstrates irregularly thickened
edema (Otake 1994; Moore and Teirstein 2003; subcutaneous tissue with several hyperechoic
Koyama etal. 2004). The peripheral areas of the plaque-like lesions. Histologically, the
nodules show increased signal intensity on hypoechoic portion corresponds to the noncase-
T1-weighted image due to the high cellularity of ating granulomas, and the hyperechoic portion
granulomas (Otake 1994). Coronal and sagittal corresponds to the surrounding inflammatory
images may show the three stripes sign, consist- infiltrates (Chen etal. 2009). Color Doppler
ing of a hypointense inner stripe and hyperintense study reveals mild hypervascularity.
outer stripes. Gallium-67 scintigraphy shows
increased uptake in the nodules but is otherwise
nonspecific (Otake 1994). US also demonstrates 16.5 Mortons Neuroma
the star-shaped central lesion and the three stripes.
However, the locations of black and white on the Mortons neuroma is a common cause of forefoot
US are reversed from their locations on MR pain. The term was popularized after the descrip-
images (Otake 1994). In myopathic type involve- tion of this condition by Thomas G.Morton in
ment, the involved muscle has nonspecific high 1876 (Bencardino etal. 2000; Murphey etal.
signal intensity on T2-weighted images. 1999). Although the exact cause is controversial,
Mortons neuroma most likely represents an monly symptomatic than are smaller ones, but
entrapment neuropathy by the overlying significant overlap was noted between the two
transverse intermetatarsal ligament, causing peri- groups (Zanetti etal. 1999; Bencardino etal.
neural fibrosis, nerve degeneration, leukocyte 2000). Therefore, careful correlation between
infiltration, and epineural and endoneural vascu- clinical and MR imaging findings is mandatory
lar hyalinization, resulting in a significantly before Mortons neuroma is considered clinically
thicker intermetatarsal nerve (Bencardino etal. relevant (Bencardino etal. 2000). The informa-
2000). Patients classically experience sharp pain, tion provided by MR imaging may have a major
a burning sensation, and paresthesias during effect on the diagnostic and therapeutic decisions
weight bearing in the region of the intermetatar- of orthopedic surgeons when Mortons neuroma
sal spaces, which can be relieved by rest. The is suspected (Zanetti etal. 1999).
third intermetatarsal space is the most frequently
involved (Torres-Claramunt etal. 2012).
Mortons neuroma is identified by US as a 16.6 Traumatic Neuroma
focal hypoechoic nodule replacing the normally
hyperechoic fat of the web spaces of the forefoot Neuroma is a nonneoplastic, insufficient, and
at the level of the metatarsal heads. In the longi- reparative proliferation of nerve tissue, the pur-
tudinal plane, Mortons neuroma appears as a pose of which is to regain axonal continuity at
fusiform hypoechoic mass, with its long axis ori- the end of an injured nerve. Neuroma is usually
ented obliquely to the metatarsals. To further observed 112months after amputation (Singson
increase diagnostic confidence and overall accu- etal. 1987; Murphey etal. 1999; Henrot etal.
racy, pressure can be applied to the medial and 2000). Typically, neuroma is associated with
lateral aspects of the forefoot. When the metatar- trauma or amputation. There are two types of
sal heads are squeezed together, Mortons neu- traumatic neuromas based on the anatomic loca-
roma abruptly displaces toward the plantar tion of the regenerating axonal mass with respect
surface of the foot, causing a palpable click, to the proximal nerve end (Singson etal. 1987;
which is the so-called sonographic Mulder sign Murphey etal. 1999; Henrot etal. 2000;
(Torriani and Kattapuram 2003). MR imaging Kransdorf and Murphey 2014). Spindle neuroma
typically demonstrates a well-defined teardrop- is a focal, fusiform swelling of the nerve away
shaped mass with intermediate-to-low signal from the severed nerve ending and represents the
intensity on T1- and T2-weighted images response of a peripheral nerve subjected to
because of its predominantly fibrous composi- chronic friction or irritation (Henrot etal. 2000).
tion. Lesions can show variable enhancement. Terminal neuroma originates at the end of the
T2-weighted image could be useful in the evalu- severed nerve and is usually due to the multidi-
ation of other diagnostic possibilities, including rectional proliferation of axons without the sup-
intermetatarsal bursitis and ganglion cyst. port of Schwann cells. This lesion has a
Plantar extension of the lesion further below the bulbous- end morphology and is continuous
level of the metatarsal head is frequently proximally with the normal nerve (Murphey
observed. In general, MR imaging shows higher etal. 1999; Henrot etal. 2000). On histological
sensitivity (76100%) than that of US in confir- analysis, traumatic neuroma shows disorganiza-
mation of the diagnosis (Zanetti etal. 1999; tion of the neurogenic tissue, which allows it to
Torres-Claramunt etal. 2012). be distinguished from neurofibroma (Murphey
There is doubt as to the necessity of imaging etal. 1999).
evidence to make this diagnosis because Mortons On US, terminal neuroma appears as a small
neuroma is frequently found on MR imaging in hypoechoic mass in continuity with the opposite
asymptomatic patients. Previous studies have edges of the severed nerve. Usually, the neuroma
demonstrated that larger (> 5mm in transverse is slightly larger than the axial diameter of the
diameter) Mortons neuromas are more com- nerve. Typically, spindle neuroma appears as a
16.7Xanthoma 295
fusiform mass with an entering and exiting nerve. terol levels. Histologically, these lesions are char-
The margins of neuromas are often well-defined, acterized by sheets of lipid-filled foamy
although some irregularity can be observed due histiocytes, extracellular cholesterol (cholesterol
to multidirectional cell proliferation (Murphey clefts), giant cells, and some inflammatory cells.
etal. 1999). On MR imaging, neuroma shows Focal cystic and degenerative change with calci-
nonspecific signal characteristics and variable fication can also be observed (Bude etal. 1994).
enhancement. However, MR imaging is the opti- On radiography, tendon xanthomas are shown
mal imaging modality to identify the direct rela- either as an abnormal tendon thickening or soft
tionship of the nerve to the lesion (Henrot etal. tissue masses without calcification (Fernandes
2000). etal. 2015). US demonstrates single or multiple
focal, hypoechoic lesions, reflecting tendon xan-
thomas or a diffusely enlarged heterogeneous
16.7 Xanthoma tendon (Bude etal. 1994). On MR imaging, a
speckled or reticulated appearance is observed on
Xanthomas are nonneoplastic lesions character- both T1- and T2-weighted images, which differs
ized by a local concentration of lipid-laden mac- from acute or chronic tears (Liem etal. 1992;
rophages, giant cells, and other inflammatory Fernandes etal. 2015). Although xanthomas are
cells in response to cholesterol deposition in tis- composed of lipids, T1 signal intensities obtained
sues. Xanthomas most frequently involve the from xanthomas are much lower than those of fat.
skin (eruptive xanthomas) and subcutaneous tis- Those MR signal characteristics are produced by
sue (tuberous xanthomas). However, the tendon, free cholesterol and cholesterol ester (the major
synovium, and, rarely, bone can also be affected lipid components of xanthomas), which do not
(Kransdorf and Murphey (2014)). Xanthomas produce a measurable MR signal. Focal areas
usually involve the extensor tendons of the hands, with a high signal on T1- and T2-weighted
both Achilles tendons, and patellar ligaments images can be observed when a component of
(tendon xanthomas) (Fernandes etal. 2015). The interfascicular edema or inflammation is present
extent of involvement is usually directly related in response to the infiltrative cholesterol deposi-
to the degree and duration of increased choles- tion. (Liem etal. 1992; Bude etal. 1994).
16.8 Illustrations: Masses That May Mimic Soft Tissue Tumors
16.8.1 Ganglion
a b
c d
Fig. 16.1 Ganglion. US (a) reveals a multilobulated first intermetatarsal space, with low signal intensity on
anechoic mass with multiple internal septa (bunch of axial T1WI (b) and high signal intensity on coronal T2WI
grapes appearance) and mild posterior acoustic enhance- (c). Coronal postcontrast FS T1WI (d) shows thin periph-
ment. The multilobulated, elongated mass is located in the eral rim enhancement
16.8 Illustrations: Masses That May Mimic Soft Tissue Tumors 297
a b
c d
Fig. 16.2 Ganglion. Axial T1WI (a) shows a well- the characteristic MR signal intensities of ganglion.
defined round mass with isointensity to adjacent skeletal Coronal postcontrast FS T1WI (d) shows thin peripheral
muscle. On axial (b) and coronal (c) T2WIs, the mass enhancement
appears with bright, high signal intensity, consistent with
a e
Fig. 16.3 Common peroneal nerve sheath ganglion cyst. lateral nerve, the affected nerve shows mild swelling and
Axial and sagittal FS T2WIs (ae) show a multilobulated increased signal intensity (arrows). There is mildly
cystic lesion (arrowheads) around the left fibula head. The increased signal intensity in anterior compartment mus-
lesion extends along the course of the common peroneal cles (d, thick arrow), suggestive of early denervation mus-
nerve (arrows), with a channel projecting anteriorly cle change
towards proximal tibiofibular joint. Compared to contra-
16.8.2 Vascular Lesion
a b
c d
Fig. 16.4 Aneurysm. Axial T1WI (a) shows a slightly enhancement of the mass, showing a characteristic flow-
hyperintense mass that is closely related to the ulnar neu- related artifact (arrows) in the direction of the phase
rovascular bundle. Axial T2WI (b) shows that the mass encoding gradient. The surgical specimen shows an
has central high and peripheral low signal intensity. enlarged mass connected with the ulnar artery (e)
Postcontrast FS T1WIs (c, d) reveals diffuse, intense
a b c
d e
Fig. 16.5 Pseudoaneurysm. Coronal T1WI (a) shows a nent pulsation artifact. CT maximum intensity projection
well demarcated ovoid mass-like lesion in the right del- image (d) shows a pseudoaneurysm (arrow) in the right
toid muscle. Coronal T1WI (a) and axial FS T2WI (b) deltoid muscle, likely supplied by the right anterior cir-
show a signal void and prominent pulsation artifact in the cumflex artery. Digital subtraction angiogram (e) reveals
direction of the phase encoding gradient. Axial postcon- extravasation of contrast (arrow) from the right anterior
trast FS T1WI (c) shows focal enhancement and a promi- circumflex artery
16.8.3 Gout
a b
c d
Fig. 16.6 Gout. There is juxta-articular soft tissue mass tellar region, involving the patellar ligament. On FS T2WI
with close proximity to the anterior cortex of the patella (c), the mass shows heterogeneous signal intensity, pre-
and involving the proximal patellar ligament (ad). dominantly intermediate to low, with overlying subcuta-
Sagittal T1WI (a) and T2WI (b) show a mass with hetero- neous fat infiltration. The mass is heterogeneously
geneous intermediate to low signal intensity in the prepa- enhanced after gadolinium administration (d)
a b
c d
Fig. 16.7 Gout. MR images (ac) shows a mass in the Radiograph of the left shoulder (d) reveals mineralization
left shoulder. On coronal T2WI (b), the mass shows het- in SASD space (arrowheads). US (e) shows a hyperechoic
erogeneous signal intensity, predominantly intermediate mass with posterior shadowing, in keeping with crystal
to low. The mass demonstrates heterogeneous enhance- deposition
ment with central cavitation on postcontrast FS T1WI (c).
16.8.4 Sarcoidosis
a b
c d e
Fig. 16.8 Subcutaneous sarcoidosis. Transverse US (a) ous infiltration with low signal intensity in the ulnar
of the forearm shows a heterogeneous echoic mass in the aspect of the forearm. Axial T2WI (d) shows a mass of
thickened subcutaneous tissue. Color Doppler study (b) intermediate signal intensity. Axial postcontrast FS T1WI
reveals several Doppler signals within the subcutaneous (e) shows that the mass has diffuse contrast enhancement
mass. Coronal T1WI (c) shows poorly defined subcutane-
a b
c d
Fig. 16.9 Muscular sarcoidosis. Axial T1WI (a) and high signal intensity. Axial postcontrast FS T1WI (d)
T2WI (b) show a slightly hyperintense nodule (arrow). reveals intense enhancement except central dark structure.
Axial T2WI (b) shows the star-shaped central structure US also demonstrates the star-shaped central lesion (e,
with dark signal intensity in the nodule (arrowhead, dark arrowhead) and the three stripes (f). However, the loca-
star sign). Coronal T2WI (c) shows three strips (arrow): tions of black and white on the US are reversed relative to
an inner stripe of low signal intensity and outer stripes of the MR images
a d
Fig. 16.10 Muscular sarcoidosis. Axial T1WI (a) shows axial (c) and sagittal (d) postcontrast FS T1WIs, a thick
a nodule of slightly increased signal intensity. Axial peripheral hyperintense rim and outer stripes show intense
T2WI (b) shows a hyperintense nodule with a central enhancement. Chest PA (e) demonstrates typical bilateral
hypointense region, exhibiting a dark star appearance. On hilar adenopathy (arrowheads)
16.8.5 Mortons Neuroma
a b
d e
Fig. 16.11 Mortons neuroma. Axial T1WI (a) obtained T1WI (c). On the longitudinal plane, US (d) shows
perpendicular to the metatarsal bones reveals a nodular teardrop-shaped hypoechoic lesion in the second inter-
mass with plantar extension (arrowhead). On axial FS metatarsal space with its long axis oriented obliquely to
T2WI (b), the lesion (arrowhead) shows lower signal the metatarsals. When squeezing the foot, transverse US
intensity compared to that of adjacent fat tissue. The mass (e) demonstrates abrupt displacement of the mass (arrow)
is not enhanced (arrowhead) on axial postcontrast FS toward the plantar surface (sonographic Mulder sign)
a b
d e
Fig. 16.12 Mortons neuroma. Axial T1WI (a) obtained shows subtle enhancement (arrowhead) on postcontrast
perpendicular to the metatarsal bones reveals a nodular FS T1WI (c). Longitudinal (d) and transverse (e) US
mass with plantar extension (arrowhead). On axial T2WI show a hypoechoic nodule (arrow) in the second web
(b), the lesion shows a lower signal intensity compared to space, replacing the normal intermetatarsal fat tissue
that of the adjacent fat tissue (arrowhead). The mass
16.8.6 Traumatic Neuroma
a b
c d
Fig. 16.13 Spindle neuroma. Axial T1WI (a) and T2WI mentioned nodule (arrow). Longitudinal US images (d, e)
(b) show a small nodule in the subcutaneous tissue of the reveal fusiform or spindle-shaped lesions with an entering
calf. Sagittal postcontrast FS T1WI (c) shows another and exiting nerve (arrows)
nodule (arrowhead) in the distal calf, below the afore-
a b
c d
Fig. 16.14 Terminal neuroma. Axial T1WI (a) and FS Coronal T1WI (d) reveals an ovoid mass at the end of the
T2WI (b) show an ovoid mass in the distal forearm of a severed median nerve (arrow). US (e) reveals a hypoechoic
patient who underwent a hand amputation. The mass is mass in continuity with the end of the severed nerve
well enhanced after gadolinium administration (c). (arrow)
a b
Fig. 16.15 Terminal neuroma. Transverse US (a) shows without the support of the Schwann cells. Longitudinal
hypoechoic terminal neuroma with a poorly defined mar- US (b) reveals hypoechoic fusiform terminal neuroma in
gin, indicating multidirectional proliferation of axons continuity with the end of the severed nerve (arrow)
16.8.7 Xanthoma
a b
c d
Fig. 16.16 Xanthoma. MR images (ac) show diffuse and T2WI (b). There is a large prepatellar mass involving
thickening of the patellar ligament and distal biceps femo- skin and subcutaneous fat tissue. Axial and sagittal post-
ris tendon. The affected tendons have a speckled appear- contrast FS T1WIs (c, d) demonstrate diffuse contrast
ance, with an inner low signal intensity on axial T1WI (a) enhancement of tendinous and subcutaneous masses
a b
c d e
Fig. 16.17 Xanthoma. Lateral radiograph of the ankle images (c, d) show diffuse thickening of Achilles tendon
(a) shows a lobulated bulging soft tissue contour in the with speckled inner low signal intensity on T1WI and
posterior aspect of the ankle. Transverse US (b) demon- T2WI, suggestive of cholesterol deposition. The ventral
strates marked diffuse thickening of the Achilles tendon, margin of the tendon has a convex outline on axial images
with a diffuse heterogeneity. Confluent hypoechoic areas (c, d). Sagittal postcontrast FS T1WIs (e) demonstrate
and thin hyperechoic foci are observed within the tendon, mild contrast enhancement of tendinous mass
causing a loss of the fibrillary pattern of tendon. MR
a b c
d e
Fig. 16.18 Xanthoma. MR images (ae) demonstrate a geneous signal intensity on T1WI (a, d) and FS T2WI (b),
thickened Achilles tendon and a large bilobed mass. The findings which are related to hemorrhagic and necrotic
mass arises from the Achilles tendon and extends from the debris within the masses. Axial (c) and sagittal (e) post-
heel to the mid-calf level. A thin rim of low signal inten- contrast FS T1WIs demonstrate mild contrast enhance-
sity (arrows) is observed on T1WI (a, d) and FS T2WI ment of tendinous mass
(b). The inner portion (stars) of the mass displays hetero-
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Part III
Practical Pearls in Diagnosis
of Soft Tissue Tumors
Lesion Characterization Based
onMR Signal Intensities 17
Most soft tissue tumors exhibit nonspecific signal chondroid tumors, vascular tumors, and vascular
intensity on MR imaging: low signal intensity on malformations. Contrast-enhanced MR images
T1-weighted images and high signal intensity on allow differentiation between truly cystic and
T2-weighted images. When a soft tissue tumor solid masses.
has a different combination of signal intensities,
it can aid in specific diagnosis and differential
diagnosis of soft tissue tumors. Tumors with high Table 17.1 T1 hyperintense lesions
signal intensity on T1-weighted images may
Lesion containing fat Lipoma
contain fat, subacute hemorrhage, high Lipoblastoma
proteinaceous fluid, or melanin (Table17.1). Fat- Hibernoma
suppression techniques can be used to distinguish Elastofibroma
fat from the other T1-hyperintense components. Fibrous hamartoma of
infancy
Intratumoral low signal intensity on T2-weighted Hemangioma
images can also be a clue to the diagnosis Liposarcoma
(Table17.2). Soft tissue tumors with collagenous Lesion containing Morel-Lavallee lesion
fibrous tissue, mineralization, hemosiderin, and methemoglobin (subacute hematoma)
Aneurysm,
vascular signal void structures can be included in
pseudoaneurysm
the differential diagnosis of the T2 hypointense Hemangioma
lesions. Occasionally, soft tissue masses have Lesion containing Ganglion
very high signal intensity similar to fluid on proteinaceous material Epidermal inclusion cyst
T2-weighted images (Table17.3). These lesions Lesion containing Malignant melanoma
include fluid cysts, bursitis, myxoid tumors, melanin Clear cell sarcoma

318 17 Lesion Characterization Based onMR Signal Intensities
Table 17.2 T2 hypointense lesions Table 17.3 T2 Fluid-equivalent hyperintense lesions

Lesion containing Calcifying aponeurotic fibroma Lesion containing Ganglion
fibrous tissue Desmoplastic fibroblastoma fluid Bursa
Fibroma of tendon sheath Epidermal inclusion cyst
Traumatic neuroma/Mortons Morel-Lavallee lesion
neuroma (fluid-like)
Granular cell tumor Lesion containing Intramuscular myxoma
Fibromatosis myxoid tissue Peripheral nerve sheath tumor
Solitary fibrous tumor Myxoid liposarcoma
Low-grade fibromyxoid sarcoma Myxofibrosarcoma
Sclerosing epithelioid Extraskeletal myxoid
fibrosarcoma chondrosarcoma
Rheumatoid nodule
Vascular lesion Hemangioma
Sarcoidosis
Lymphangioma
Lesion containing Myositis ossification Glomus tumor
mineralization Hemangioma (phlebolith)
Lesion containing Soft tissue chondroma
Hemangioma (ossifying)
cartilage
Pilomatricoma
Soft tissue chondroma
Extraskeletal osteosarcoma
Gout
Lesion containing Tenosynovial GCT
hemosiderin Hemangioma (thrombus)
Lesion containing Xanthoma
cholesterol
Lesion containing Hibernoma
high-flow vessels Solitary fibrous tumor
Vascular lesions including
arteriovenous malformation and
hemangioendothelioma
Alveolar soft part sarcoma
Aneurysm, pseudoaneurysm
17.1 T1 Hyperintense Lesions
17.1.1 Lesion Containing Fat
Sag T1
Fig. 17.1Lipoma
Ax T1 CT
Fig. 17.2Lipoblastoma
Cor T1 Ax T2
Fig. 17.3Hibernoma
Ax T1 Ax T2
Fig. 17.4Elastofibroma
Ax T1 Ax T2
Fig. 17.5 Fibrous hamartoma of infancy
Sag T1 Ax T2
Fig. 17.6Hemangioma
Ax T1 Ax T2
Fig. 17.7Liposarcoma
17.1.2 Lesion Containing Methemoglobin
Cor T1 Ax T2
Ax T1
Fig. 17.8 Morel-Lavallee lesion (hematoma)
Cor T1 Ax T1
Fig. 17.9 Aneurysm, pseudoaneurysm

17.1.3 Lesion Containing Proteinaceous Material
Ax T1 Ax T2
Fig. 17.10 Epidermal inclusion cyst
Ax T1 Ax FS T2
Fig. 17.11Ganglion
17.1.4 Lesion Containing Melanin
Fig. 17.12Malignant Cor T1 Cor C+ FS T1

melanoma
Cor T1 Cor C+ FS T1
Fig. 17.13 Clear cell sarcoma

17.2 T2 Hypointense Lesions
17.2.1 Lesion Containing Fibrous Tissue
Ax T2
Fig. 17.14 Calcifying aponeurotic fibroma
Ax FS T2 Ax C + FS T1
Fig. 17.15 Desmoplastic fibroblastoma

Sag T2 Sag C + FS T1
Fig. 17.16 Fibroma of tendon sheath
Ax T2 US
Fig. 17.17 Traumatic neuroma/Mortons neuroma

Cor FS T2 Ax C+ FS T1
Fig. 17.18 Granular cell tumor
Ax T2 Ax C + FS T1
Fig. 17.19Fibromatosis
Ax T2 Ax C+ FS T1
Fig. 17.20 Solitary fibrous tumor
Ax T2 Ax C + T1
Fig. 17.21 Low-grade fibromyxoid tumor
Cor T2 Cor C+ FS T1
Fig. 17.22 Rheumatoid nodule

Fig. 17.23Sarcoidosis Ax T2 Cor T2
17.2.2 Lesion Containing Mineralization
Ax T2
Fig. 17.24 Myositis ossification

Fig. 17.25Hemangioma
Sag FS T2
(phlebolith)
Fig. 17.26Ossifying Cor T1

hemangioma
Fig. 17.27Pilomatricoma Ax T2 Ax T1
Sag FS T2
Fig. 17.28 Soft tissue chondroma
Pelvis AP Ax T2
Fig. 17.29 Extraskeletal osteosarcoma

Cor T2 Cor C+ FS T1
Fig. 17.30Gout
Fig. 17.31 Tenosynovial GCT
Ax T2 Ax C + FS T1
Fig. 17.32 Hemangioma (thrombus)

17.2.3 Lesion Containing Free Cholesterol
Fig. 17.33Xanthoma Ax T1 Ax T2
17.2.4 Lesion Containing High-Flow Vessels
Cor T1 Cor C + FS T1
Fig. 17.34Hibernoma
Cor FS T2 Cor C + FS T1
Fig. 17.35 Solitary fibrous tumor
Cor T1 Ax T2
Fig. 17.36 Vascular lesions including arteriovenous malformation and hemangioendothelioma

Fig. 17.37Alveolar
soft part sarcoma
Cor T2 Ax T1
Fig. 17.38 Aneurysm, pseudoaneurysm

17.3 T2 Fluid-Equivalent Hyperintense Lesions
17.3.1 Lesion Containing Fluid
Ax FS T2 Ax C+ FS T1
Fig. 17.39Ganglion
Ax T2 Sag T1
Fig. 17.40Bursitis
Ax T2 Ax C + FS T1
Fig. 17.41 Epidermal inclusion cyst
Ax T2 Ax T1
Ax C + FS T1 (subtraction)
Fig. 17.42 Morel-Lavallee lesion (fluid-like)

17.3.2 Lesion Containing Myxoid Tissue
Fig. 17.43 Intramuscular myxoma
Fig. 17.44Peripheral Sag T2 Ax C + FS T1

nerve sheath tumor
Cor FS T2 Cor T1
Fig. 17.45 Myxoid liposarcoma
Ax T2 Ax C + FS T1
Fig. 17.46Myxofibrosarcoma
Fig. 17.47 Extraskeletal myxoid chondrosarcoma

17.3.3 Vascular Lesion
Fig. 17.48Hemangioma
Ax T2 Ax C + FS T1 (subtraction)
Fig. 17.49Lymphangioma
Fig. 17.50 Glomus tumor

17.3.4 Lesions Containing Cartilage
Fig. 17.51 Soft tissue

Sag FS T2 Ax C + FS T1
chondroma
Diagnostic Signs
18
18.1 Bowl ofGrapes Sign Multilocular mass with fluidfluid levels

Seen in synovial sarcoma
Heterogeneous signal intensity
Ax T1 Ax T2
Sag FS T2
Fig. 18.1

346 18 Diagnostic Signs
18.2 Bunch ofGrapes Appearance
Multilobulated anechoic mass with multiple

internal septa on US
Seen in ganglion
Cor FS T2
US
Cor FS T2 US
Fig. 18.2
18.3Checkerboard Appearance 347
18.3 Checkerboard Appearance Hyperintense strands between hypointense

muscle fascicles on T2WI and postcontrast
Checkerboard pattern resembling dry, cracked T1WI
mud Seen in proliferative myositis
Ax T1 Ax T2
Ax C + FS T1
Fig. 18.3
18.4 Coaxial Cable-Like

Appearance
Serpentine nerve fascicles embedded within

fatty tissue
Seen in lipomatosis of nerve in axial MRI
Ax T1 Ax C+ FS T1
Ax T1 Ax T1
Fig. 18.4
18.5Dark Star Sign 349
18.5 Dark Star Sign Star-shaped central structure with increased

echotexture on US
Star-shaped central structure with low signal Seen in sarcoidosis
intensity on MRI
Ax T1 Ax T2
Ax C+ FS T1 US
Fig. 18.5
18.6 Fascicular Sign Reflecting the fascicular bundles within the

nerves seen histologically
Multiple small hypointense ringlike structures Found in peripheral nerve sheath tumors (neu-
within the hyperintense area on T2WI rofibroma, schwannoma)
Cor FS T2 Ax T2
Cor T2 Cor T2
Fig. 18.6
18.7Inverted Target Sign 351
18.7 Inverted Target Sign Non-enhancing central area on postcontrast

T1WI
Central T2 hyperintensity and peripheral T2 Seen in nodular fasciitis
hypointensity
Ax T2 Ax C+ FS T1
Cor FS T2 Cor C+ FS T1
Fig. 18.7
18.8 Gyriform Pattern Low to iso-signal intensity on fluid-sensitive

sequences
Multiple folded layers mimicking brain gyri Seen in low-grade fibromyxoid sarcoma
Cor T1 Cor T2
Cor C+ FS T1 Ax T2
Fig. 18.8
18.9Reverse Target Sign 353
18.9 Reverse Target Sign surrounding myxoid component on postcon-

trast T1WI
Enhancement of the central fibrous compo- Seen in peripheral nerve sheath tumors (neu-
nent and a relative lack of enhancement of the rofibroma, schwannoma)
Ax T2 Ax C+ FS T1
Fig. 18.9
18.10 Reverse Zoning Pathological findings of a reverse zoning

Phenomenon phenomenon: central deposition of osteoid
material and atypical spindle cell proliferation
Central location of the osteoid in the lesion at the periphery
The densest ossification (arrow) in the center Found in extraskeletal osteosarcoma
of the lesion and the least ossification at the
periphery
Cor FS T2
Fig. 18.10
18.11Spaghetti-Like Appearance 355
18.11 Spaghetti-Like Appearance fatty tissue

Seen in lipomatosis of nerve on longitudinal
Serpentine nerve fascicles embedded within MRI
Cor T1 Cor T1
Fig. 18.11
18.12 Split Fat Sign Best seen on T1WI

Observed in peripheral nerve sheath tumors
Tapered rim of fat at the upper and lower poles and other soft tissue tumors in intermuscular
of a lesion or fine rind of the fat around the location
lesion
Sag T1 Cor T1
Cor T2 Cor T1
Fig. 18.12
18.13String Sign 357
18.13 String Sign Delineation of the entering and exiting nerve

in a lesion, affecting large, deep nerves
On US and MRI Seen in peripheral nerve sheath tumor
Sag FS T2 Sag FS T2
Sag T2 US
Fig. 18.13
18.14 Stripe Sign with the adjacent muscle fibers at the cranio-
caudal portion of the mass, representing the
On US and MRI preservation of the adjacent muscle fibers
Intralesional hypointense lines continuous Described in granular cell tumor
Cor T2 US
Fig. 18.14
18.15Swiss Cheese Appearance 359
18.15 Swiss Cheese Appearance on T1WI and T2WI, corresponding to the

ossification
X-ray: multiple rings and arc-like ossification Seen in ossifying hemangioma
with a coarse trabecular pattern Different from zonal phenomenon in myositis
MRI: non-enhanced, spotted, or irregular ossificans
shaped areas of very low signal intensity both
Cor FS T2
Sag FS T2
Fig. 18.15
18.16 Tail Sign Tapered fascial enhancement extending from

the tumor margin with more than 2 mm thick-
Tail-like multidirectional spread along the fas- ness on postcontrast T1WI
cial plane on T2WI Observed in undifferentiated pleomorphic sar-
coma, myxofibrosarcoma, and nodular fasciitis
Ax T2 Ax T1
Ax C+ FST1
Fig. 18.16
18.17Target Sign 361
18.17 Target Sign Surrounding area of high signal intensity:

more myxomatous tissue
On T2WI Seen in peripheral nerve sheath tumors (neu-
Central area of low signal intensity: histologi- rofibroma, schwannoma)
cally fibrocollagenous tissue in the tumors core
Ax T2 Sag FS T2
Ax T2 Sag T2
Fig. 18.17
18.18 Three Stripes Sign Reversed locations of black and white on US

Seen in sarcoidosis
Three strips, an inner stripe of low signal
intensity and outer stripes of high signal inten-
sity on T2WI and postcontrast FS T1WI
Cor T2 Sag C + FS T1a
US
Fig. 18.18
18.19Triple Signal Sign 363
18.19 Triple Signal Sign Intermediate SI: solid cellular elements

High SI: hemorrhage or necrosis
Areas of high, intermediate, and low signal Seen in synovial sarcoma
intensity (SI) on T2WIs
Low SI: calcified, hemorrhagic, or fibrotic
collagenized regions
Ax T1 Ax T2
Ax C + FS T1 CT
Fig. 18.19
18.20 Zonal Phenomenon Can be better appreciated on CT than MRI

Seen in myositis ossificans
Peripheral mineralization and non-mineralized
center
Cor FS T2 Ax T2
CT
Fig. 18.20
Syndrome-Associated Soft Tissue
Tumors 19
Soft tissue neoplasms may be associated with a mary of syndromes, associated soft tissue tumors,
variety of genetic disorders and malformation which are uncommon, but not rare, is shown in
syndromes, especially when they arise in chil- Table19.1.
dren, adolescents, and early adulthood. A sum-
Table 19.1 Syndromes and associated soft tissue tumors

Syndrome Soft tissue tumors Principal features
Kasabach-Merritt syndrome Kaposiform hemangioendothelioma Microangiopathic hemolytic
Infantile hemangioma anemia
Tuft angioma Thrombocytopenia
Maffucci syndrome Hemangioma Enchondromas
Lymphangioma Chondrosarcoma
Epithelioid hemangioendothelioma
Klippel-Trenaunay syndrome Venous or lymphatic malformation Nevus inflammeus
Hypertrophy of associated bone
and soft tissue
Mazabraud syndrome Intramuscular myxoma Fibrous dysplasia
Neurofibromatosis type 1 Neurofibromas Caf au lait spots
MPNST Lisch nodules
Neurofibromatosis type 2 Schwannoma Meningioma
Neurofibroma Astrocytoma
Rarely perineurioma
Schwannomatosis Schwannomas Without vestibular nerve
involvement
Carney complex Melanotic schwannoma Involvement of multiple
Myxoma endocrine glands
Pigmented lesions of the skin and
mucosa
Familial hypercholesterolemia Xanthomas High cholesterol and LDL level

366 19 Syndrome-Associated Soft Tissue Tumors
19.1 Kasabach-Merritt Syndrome Thrombocytopenia

Consumptive coagulopathy
Rapidly growing vascular tumor: kaposiform
hemangioendothelioma, infantile hemangi-
oma, tuft angioma
a b
Fig. 19.1 Kasabach-Merritt syndrome. Infantogram (a) consistent with lymphangioma in the left axilla. MR angi-
shows a huge soft tissue mass or hypertrophy in the left ography (e) better demonstrates the extent of the enhanc-
shoulder. Coronal T1WI (b), T2WI (c), and postcontrast ing hemangiomatous lesions (arrows). Seven years later,
FS T1WI (d) show diffuse hemangiomatous involvement radiograph (f) and coronal postcontrast FS T1WI (g) show
of the soft tissues in the left shoulder, axilla, and upper a marked decrease in extent of the soft tissue lesions. The
arm. There is a non-enhancing component (arrow in d) patient had received radiation therapy
19.1 Kasabach-Merritt Syndrome 367
e f
19.2 Maffucci Syndrome Multiple enchondromas

Soft tissue vascular lesions: cavernous or cap-
Congenital, nonhereditary mesodermal illary hemangiomas, lymphangiomas, epithe-
dysplasia lioid hemangioendotheliomas
b c d
Fig. 19.2 Maffucci syndrome. AP radiograph of both (c), and postcontrast FS T1WI (d) show multiple soft tis-
hands (a) shows multiple enchondromas in the left 3rd sue hemangiomas in the right wrist and thumb. Lateral
5th fingers and the left distal radius and ulna. There are radiograph of the left knee (e) shows multiple channel-
multiple soft tissue masses in the right wrist and thumb, like enchondromas in the left femur and tibia. Sagittal (f)
one (arrow) of which has multiple calcific foci and erodes and coronal (g) FS T2WIs show multiple enchondromas
the trapezium and scaphoid. Coronal T1WI (b), FS T2WI of the bone and soft tissue hemangiomas (arrows)
19.2 Maffucci Syndrome 369
e f
19.3 Klippel-Trenaunay Venous or lymphatic malformations

Syndrome Soft tissue hypertrophy
Nevus inflammeus
a b
Fig. 19.3 Klippel-Trenaunay syndrome. Coronal (a) and left lower extremity. Axial T1WI (d) demonstrates subcu-
axial (b, c) CT images show hypertrophied soft tissues in taneous and muscular hypertrophies in the left thigh along
the left lower extremity. There are multiple varicose veins with intramuscular venous malformation (arrow)
and intramuscular venous malformation (arrows) in the
19.4 Mazabraud Syndrome 371
19.4 Mazabraud Syndrome Multiple soft tissue myxomas
Fibrous dysplasia (usually polyostotic)
a b
c d
Fig. 19.4 Mazabraud syndrome. AP radiograph of the postcontrast FS T1WI (d) show multilobulated intramus-
pelvis (a) shows multiple fibrous dysplasia in the pelvis cular myxomas in the both buttocks (arrows) and fibrous
and the right femur. Coronal T1WI (b), T2WI (c), and dysplasia in both ilia (arrowheads)
19.5 Neurofibromatosis Type 1 Vertebral scalloping, enlarged neural foram-

ina: due to dural ectasia or neurofibromas
Caf au lait spots Deural ectasia
Iris hamartomas (Lisch nodules) Ribbon rib deformity, rib notching, and
Associated soft tissue tumors: neurofibomas dysplasia
with increased risk of MPNST (malignant Cortical thinning, erosive defects, sclerosis,
peripheral nerve sheath tumor) and periosteal proliferation due to extrinsic
Musculoskeletal manifestation pressures
Plexiform neurofibromas Pseudoarthrosis and bowing: related to
Kyphoscoliosis mesodermal dysplasia
a b
c d
Fig. 19.5 Neurofibromatosis type 1. Chest radiograph sions onto the neighboring iliac bones. Both femoral neck
(a) shows several rib notching or mild ribbon rib defor- deformities are not associated with neurofibomas but
mity (arrowheads). AP radiograph of the pelvis (b) dem- related to the skeletal dysplasia. Multiple, variable-sized,
onstrates marked deformities of pelvic bones and both circumscribed, localized neurofibromas (thin arrows) are
femora such as cortical thinning, erosive defects, sclero- noted in the subcutaneous tissue, muscle, and fascial
sis, and periosteal proliferation. Coronal FS T2WIs (c, d) planes. In addition, infiltrative, diffuse neurofibromas
show neurofibromas (arrows) with marked pressure ero- (stars) involving multicompartments are shown
a b c
Fig. 19.6 Neurofibromatosis type 1. Whole spine stand- peroneal nerve and its branches. The target sign with low
ing AP radiograph (a) shows a thoracic scoliosis. signal centrally and high signal intensity peripherally is
Teleradiograph of the lower extremity (b) reveals dysplas- seen in numerous lesions. Two years later, he complained
tic left proximal tibia with irregular thick cortex and wid- of local pain and swelling at the proximal tibial level.
ening of the affected tibia. Coronal FS T2WI (c) shows Follow-up sagittal T2WI (e) shows interval development
multiple, circumscribed, oval hyperintense soft tissue of a large subcutaneous mass (star). The mass has isoin-
lesions (thin arrows) along the nerve courses in both tense to muscle on T1WI (f) and heterogeneous hyperin-
thighs. Sagittal FS T2WI (d) of the lower leg shows a tensity on T2WI (g) with inhomogeneous enhancement
typical appearance, diffuse thickened nerve with nodular- on postcontrast FS T1WI (h). These findings represent
ity, of plexiform neurofibroma affecting the common malignant transformation
d e
f g h
a b
Fig. 19.7 Neurofibromatosis type 1. Coronal T2WI of dural sac (dural ectasia) with direct extension into the
the thoracic spine (a) shows the scoliosis with hemiverte- paravertebral space (star) through the marked widened
bra (arrow). Axial T2WI (b) demonstrates the dilated left neural foramen
Fig. 19.8Neurofibromatosis
type 1. AP radiograph of the a b
right forearm (a) shows a
dysplastic appearance of the
affected radius and ulna with
narrowing, sclerosis, and
obliteration of the medullary
canal (pseudoarthrosis). AP
radiograph of the left forearm
(b) for comparison
19.6 Neurofibromatosis Type 2 Meningiomas and other CNS tumors
Bilateral acoustic schwannomas and other

schwannomas
a b
c d
Fig. 19.9 Neurofibromatosis type 2. Axial T2WIs of the axial (i) T2WIs reveal two dark signal intradural extra-
brain shows bilateral trigeminal (a) and acoustic (b) medullary masses (arrows) at the level of C1 and T1/2,
schwannomas (arrows). T2WI (c) and CT (d) show a respectively, which are pressing the spinal cord and keep
large calcifying left meningioma abutting the falx. Sagittal in with calcified meningiomas. In addition, there are
(e) and axial (h) T2WIs demonstrate a extradural multi- numerous tiny leptomeningeal nodules (thin arrows)
lobulating, circumscribed soft tissue mass (star) with along the cord surface and cauda equina, representing
pressure erosions, and resultant neural foraminal widen- schwannomatosis
ing, which represents a schwannoma. Sagittal (f, g) and
e f g
h i
a b
Fig. 19.10 Neurofibromatosis type 2. Coronal FS (a) mediate signal area and thin peripheral hyperintense rim,
and axial (b) T2WIs of the knee show aggregation of the respectively. Brain MRI (c) of the same patient shows
variable-sized, circumscribed soft tissue lesions affecting bilateral acoustic schwannomas (arrows)
the skin and subcutaneous tissue, which has central inter-
19.7Schwannomatosis 379
19.7 Schwannomatosis Only 15%: familial

Recognized most often in people over the age
Known as neurilemmomatosis of 30
Multiple schwannomas without vestibular
nerve involvement or meningioma
a b c d
Fig. 19.11 Schwannomatosis. Sagittal FS T2WI (a) of neous intense enhancing soft tissue lesions (arrowheads)
right upper arm reveals two circumscribed oval soft tissue along the nerve course. Two of those were surgically con-
masses (arrows) with target and string signs. Coronal firmed as schwannomas
postcontrast FS T1WIs (b, c, d) show multiple inhomoge-
19.8 Carney Complex Pigmented lesions of the skin and mucosa

Associated soft tissue tumors: melanotic
Multiple neoplasia syndrome schwannomas, myxomas
Involvement of multiple endocrine glands
a b
c d e
Fig. 19.12 Carney complex. A 33-year-old male has the contrast FS T1WI (e). Hyperintensity on T1WI represents
history of Cushing syndrome, s/p adrenalectomy, and car- melanin deposition. Sagittal MRI shows the C5 lesion to
diac myxoma. Lateral radiograph of the cervical spine (a) have the same signal characteristics as the above soft tis-
reveals an osteolytic lesion (arrow) affecting C5 vertebral sue mass. Note a smaller intraosseous lesion (thin arrows)
body with pathologic fracture. PET (b) demonstrates sev- in C3 spinous process, which shows mildly hypointensity
eral hypermetabolic bony lesions (arrowheads) at the cer- on T2WI (f) and T1WI (g), and diffuse enhancement on
vical and thoracolumbar junction, suggesting bone postcontrast FS T1WI (h). Reactive prevertebral soft tis-
metastases. Axial MRI of the cervical spine shows a mul- sue edema (star) is shown. CT-guided bone biopsy was
tilobulating soft tissue mass (arrowheads) C3/4 right neu- performed for T12 vertebral lesion, and then melanotic
ral foramen with direct extension into intradural space and schwannoma was confirmed. Finally, melanotic schwan-
mild cord compression, which has dark signal intensity on noma (arrowheads) C3/4 right neural foramen and multi-
T2WI (c), hyperintensity on T1WI (d), and mild hetero- ple bone metastases were diagnosed
geneous enhancement such as rim enhancement on post-
19.8 Carney Complex 381
f g h
19.9 Familial blood

Hypercholesterolemia Yellow deposits of cholesterol-rich fat in the
skin, and tendons, particularly the Achilles
High cholesterol levels, specifically very high tendon
levels of low-density lipoprotein (LDL), in the
a b
Fig. 19.13 Familial hypercholesterolemia. MR images T2WI (a) and US (eg) also demonstrate skin and sub-
show hypointense soft tissue masses (tendinous xantho- cutaneous masses (tuberous xanthomas) on the extensor
mas) in patellar ligament (a), biceps femoris tendon (b), surface of knee (a), elbow (e), distal forearm (f), and
Achilles tendon (c), and flexor carpi ulnaris (d). Axial finger (g)
19.9 Familial Hypercholesterolemia 383
f g
Part IV
Drill and Practice
Image Interpretation Session
20

388 20 Image Interpretation Session
20.1 Quiz
A 54-year-old male, left posterior upper arm

mass
CorT1 Ax T1
Fig. 20.1
20.1Quiz 389
20.1 Answer
Cor T1 Ax T1
Myxoid liposarcoma (Chap. 4)

Small amount of fat in septa (arrows)
Marked hyperintensity on T2WI
Myxoid-pattern enhancement
20.2 Quiz
A 83-year-old female, left upper arm palpable mass
Cor T1 Cor FS T2
Cor C+ FS T1 Ax T2
Fig. 20.2
20.2Quiz 391
20.2 Answer
Cor T1 Cor FS T2
Cor C+ FS T1 Ax T2
Schwannoma (Chap. 12)

Entering radial nerve (arrowhead)
Fascicular sign on T2WI (small arrows)
Split-fat sign on T1WI (arrows)
20.3 Quiz
A 58-year-old female, right knee painful mass
Ax T1 Ax T2
Sag FS T2 Sag C+ FS T1
Fig. 20.3
20.3Quiz 393
20.3 Answer
Ax T1 Ax T2
Sag FS T2 Sag C+ FS T1
Tenosynovial giant cell tumor, diffuse type

(Chap. 6)
Intra-articular lesion location
Very low signal regions (arrowheads) in the
lesion on all sequences, reflecting hemosid-
erin depositions
20.4 Quiz
An 11-year-old male, right pubic mass
Ax T1 Cor C+ FS T1
Ax T2
CT
Fig. 20.4
20.4Quiz 395
20.4 Answer
Ax T1 Cor C+ FS T1
Ax T2
CT
Myositis ossificans (Chap. 5)

Slightly T1 hyperintense mass
Surrounding soft tissue edema and enhance-
ment (arrows) on T2WI and postcontrast
T1WI
Zonal phenomenon of calcification (arrow)
onCT
20.5 Quiz
A 47-year-old female, left wrist mass
Cor T1 Cor FS T2
Ax FS T1 Cor C+ FS T1
Fig. 20.5
20.5Quiz 397
20.5 Answer
Cor T1 Cor FS T2
Ax FS T1 Cor C+ FS T1
Ganglion (Chap. 16)

Fluid-like high signal intensity on T2WI
Multilobulated and elongated appearance on
T2WI
No contrast enhancement
20.6 Quiz
A 67-year-old male, right upper arm mass
Cor FS T2 Ax FS T2
Ax C+ FS T1
Fig. 20.6
20.6Quiz 399
20.6 Answer
Cor FS T2 Ax FS T2
Ax C+ FS T1
Proliferative myositis (Chap. 5)

Expansile muscle mass with preserved
fascicles
Extensive perilesional edema (arrows)
Checkerboard appearance on axial T2WI and
postcontrast T1WI
20.7 Quiz
A 48-year-old female, right thigh mass
Sag T2
Ax T2
Ax T1 Ax C+ FS T1
Fig. 20.7
20.7Quiz 401
20.7 Answer
Sag T2
Ax T2
Ax T1 Ax C+ FS T1
Intramuscular myxoma (Chap. 13)

Fluid-like bright high signal intensity on
T2WI
Fuzzy and mild contrast enhancement
20.8 Quiz
A 47-year-old female, left wrist mass
Cor T1 Ax FS T2
Cor C+ FS T1
Ax C+ FS T1
Fig. 20.8
20.8Quiz 403
20.8 Answer
Cor T1 Ax FS T2
Cor C+ FS T1
Ax C+ FS T1
Tendinous xanthoma (Chap. 16)

Enlargement of flexor carpi ulnaris tendon
with speckled inner low signal intensity on
T1- and T2WI
Diffuse contrast enhancement
20.9 Quiz
A 43-year-old male, finger mass 1year ago
Ax T1
Ax T2
Ax C+ T1 (Subtraction)
Fig. 20.9
20.9Quiz 405
20.9 Answer
Ax T1
Ax T2
Ax C+ T1 (Subtraction)
Thrombosed hemangioma (Chap. 10)

Phleboliths (arrowheads)
Diffuse intralesional hyperintensity on T1WI
and hypointensity on T2WI, representing
thrombi (arrows)
No demonstrable contrast enhancement on the
subtracted postcontrast T1WI
20.10 Quiz
A 66-year-old female, right subdeltoid bursa

lesion
Cor T1 Cor FS T2
Fig. 20.10
20.10Quiz 407
20.10 Answer
Cor T1 Cor FS T2
Lipoma arborescens (Chap. 4)

Frond-like synovial mass
Isointense (arrows) to fat on T1WI
Suppressed signal on fat suppression sequence
20.11 Quiz
A 70-year-old male, right lower leg mass
Ax T1 Ax FS T2
Ax C+ FS T1
Fig. 20.11
20.11Quiz 409
20.11 Answer
Ax T1 Ax FS T2
Ax C+ FS T1
Myxofibrosarcoma (Chap. 5)
Perifascial mass with infiltrative margin
Curvilinear projection along fascial plane,
tail sign (arrows)
20.12 Quiz
A 2-year-old boy, right arm mass
Ax T2
Ax C+ FS T1
Cor T1
Fig. 20.12
20.12Quiz 411
20.12 Answer
Ax T2
Ax C+ FS T1
Cor T1
Lymphangioma (Chap. 10)

Child
Huge subcutaneous non-enhanced cystic mass
(stars)
20.13 Quiz
A 37-year-old male, right thigh mass
Ax T1 Ax T2
Cor T1 Ax C+ FS T1 (Subtraction)
Fig. 20.13
20.13Quiz 413
20.13 Answer
Ax T1 Ax T2
Cor T1 Ax C+ FS T1 (Subtraction)
Morel-Lavallee lesion (Chap. 15)

High signal intensity on T1WI
Dark signal rim on T2WI
No contrast enhancement except peripheral
thin wall on postcontrast FS T1
20.14 Quiz
A 55-year-old female, right posterior shoulder

mass
Cor T1 Sag T2
Fig. 20.14
20.14Quiz 415
20.14 Answer
Cor T1 Sag T2
Hibernoma (Chap. 4)
Slightly hypointense relative to fat on T1WI
Incomplete signal suppression on FS T2WI
Prominent internal vascularity (arrows)
20.15 Quiz
A 36-year-old male, left anterior elbow mass
Sag T2 Ax T1
Ax C+ FS T1
Fig. 20.15
20.15Quiz 417
20.15 Answer
Sag T2 Ax T1
Ax C+ FS T1
Dedifferentiated liposarcoma (Chap. 4)

Well-differentiated liposarcoma-type compo-
nent (stars)
Enhancing nonlipomatous mass (arrow)
20.16 Quiz
A 79-year-old male, right medial foot pain
Cor T1 Cor T2 Cor C+ FS T1
Fig. 20.16
20.16Quiz 419
20.16 Answer
Cor T1 Cor T2 Cor C+ FS T1
Chronic tophi in gout (Chap. 16)

Low signal intensity on T1WI and T2WIs
(arrows)
Only peripheral enhancement
Calcific deposit (arrowhead)
20.17 Quiz
A 19-year-old female, left medial thigh mass
Ax T1 Ax C+ FS T1
Sag T2 Sag C+ FS T1
Fig. 20.17
20.17Quiz 421
20.17 Answer
Ax T1 Ax C+ FS T1
Sag T2 Sag C+ FS T1
Alveolar soft part sarcoma (Chap. 13)

Multiple signal voids in and around the mass
on T2WI (arrows)
Strong enhancement with surrounding signal
voids
20.18 Quiz
A 56-year-old female, left posterior thigh mass
Ax T1 Ax T2
Ax C+ FS T1
Fig. 20.18
20.18Quiz 423
20.18 Answer
Ax T1 Ax T2
Ax C+ FS T1
Well-differentiated liposarcoma (Chap. 4)

Large fatty mass
Multiple thick and enhancing septa (arrows)
No significant nonlipomatous component
20.19 Quiz
A 27-year-old male with neurofibromatosis type

1, growing painful lower leg mass
Sag FS T2 Ax T2
Ax C+ FS T1
Fig. 20.19
20.19Quiz 425
20.19 Answer
Sag FS T2 Ax T2
Ax C+ FS T1
Malignant peripheral nerve sheath tumor

(Chap. 12)
Neurofibromatosis patient
Large size of the lesion
Intralesional hemorrhagic necrosis (arrows)
Extensive surrounding perilesional edema
(arrowheads)
20.20 Quiz
A 71-year-old male, right chest wall swelling
Ax CT
US
PET-CT
Fig. 20.20
20.20Quiz 427
20.20 Answer
Ax CT
US
PET-CT
Lymphoma (Chap. 15)

Diffuse muscle swelling on CT and US
Strong FDG uptake on PET-CT
20.21 Quiz
A 39-year-old male, left hand mass
Ax T1 Ax T2
Ax C+ FS T1
Fig. 20.21
20.21Quiz 429
20.21 Answer
Ax T1 Ax T2
Ax C+ FS T1
Aneurysm (Chap. 16)

Internal dark signal portion on T2WI
Prominent pulsation artifact (arrows) around
the mass on phase-encoding direction
20.22 Quiz
A 22-year-old male, right elbow mass
Ax T1 Ax T2
US
Fig. 20.22
20.22Quiz 431
20.22 Answer
Ax T1 Ax T2
US
Pilomatricoma (Chap. 15)

Low signal intensity on T1- and T2WIs
(arrowheads)
Linear hyperechoic lesion with posterior
shadowing on US
20.23 Quiz
A 69-year-old female, slowly growing thigh mass
Sag FS T2
Ax T1 Ax C+ FS T1
Fig. 20.23
20.23Quiz 433
20.23 Answer
Sag FS T2
Ax T1 Ax C+ FS T1
Extraskeletal osteosarcoma (Chap. 11)

Intralesional haphazard osteoid mineraliza-
tion (arrowheads)
20.24 Quiz
A 65-year-old male, left posterior shoulder mass
Cor T1 Sag FS T2
Sag C+ FS T1
Fig. 20.24
20.24Quiz 435
20.24 Answer
Cor T1 Sag FS T2
Sag C+ FS T1
Desmoplastic fibroblastoma (Chap. 5)

Predominantly low signal on T1- and T2WIs
(arrow)
Non-enhancing collagenous components (arrow)
20.25 Quiz
A 7-year-old boy, right lateral knee mass
Sag FS T2 Sag FS T2 Sag FS T2
Fig. 20.25
20.25Quiz 437
20.25 Answer
Intraneural ganglion (Chap. 16)

Elongated mass along the common peroneal
nerve with fluid-like high signal intensity on
T2WI (arrows)
20.26 Quiz
A 60-year-old male, right shoulder mass
Ax T1 Ax T2
Ax C+ FS T1
Fig. 20.26
20.26Quiz 439
20.26 Answer
Ax T1 Ax T2
Ax C+ FS T1
Extraskeletal myxoid chondrosarcoma

(Chap. 13)
High signal intensity with thin fibrous septa of
low signal intensity on T2WI
An area of necrotic change (arrow)
Prominent enhancement with peripheral/sep-
tal enhancement pattern
20.27 Quiz
A 31-year-old male, left thigh palpable mass
Cor T1
Ax T2 Ax C+ FS T1
Fig. 20.27
20.27Quiz 441
20.27 Answer
Cor T1
Ax T2 Ax C+ FS T1
Ossifying hemangioma (Chap. 10)

Swiss cheese appearance of intralesional
mineralization (arrowheads)
Periosteal cortical hypertrophy (arrow)
Peripheral fat overgrowth (small arrows)
20.28 Quiz
A 65-year-old male, right thigh mass
Ax T1 Ax T2
Cor T1 Sag FS T1
Fig. 20.28
20.28Quiz 443
20.28 Answer
Ax T1 Ax T2
Cor T1 Sag FS T1
Terminal neuroma (Chap. 16)

Hypointense mass (arrowheads) on T1- and
T2WIs
A mass in continuity with the end of the sev-
ered sciatic nerve (arrows)
20.29 Quiz
A 12-year-old girl, slowly growing foot mass
Ax T1 Ax FS T2
Ax C+ FS T1 Sag FS T2
Fig. 20.29
20.29Quiz 445
20.29 Answer
Ax T1 Ax FS T2
Ax C+ FS T1 Sag FS T2
Tenosynovial giant cell tumor, localized type

(Chap. 6)
Very low signal regions (arrowheads) within
the lesion on T2WI, reflecting hemosiderin
depositions
20.30 Quiz
A 32-year-old female, pain and cold sensitivity
Ax T1
Ax FS T2
Ax FS T1
Cor C+ FS T1
Fig. 20.30
20.30Quiz 447
20.30 Answer
Ax T1
Ax FS T2
Ax FS T1
Cor C+ FS T1
Glomus tumor (Chap. 8)
Located in the nail bed

Present neighboring bone erosion (arrowheads)
Intense contrast enhancement due to hyper-
vascularity (star)
20.31 Quiz
A 25-year-old male, neurofibromatosis type 1
Ax T1 Ax T2
Ax C+ FS T1
Fig. 20.31
20.31Quiz 449
20.31 Answer
Ax T1 Ax T2
Ax C+ FS T1
Neurofibroma, diffuse type (Chap. 12)
Neurofibromatosis type 1
Infiltrative soft tissue mass affecting multiple
tissue planes (arrows)
20.32 Quiz
A 42-year-old male, left posterior elbow mass
Sag FS T2 Ax T1
Ax C+ FS T1
Fig. 20.32
20.32Quiz 451
20.32 Answer
Sag FS T2 Ax T1
Ax C+ FS T1
Fibromatosis (Chap. 5)
Infiltrative soft tissue mass
Marked contrast enhancement
T2 hypointense, non-enhancing band-like
components (arrows)
20.33 Quiz
An 1-year-old boy, left thigh mass
Cor T1 Ax T1
Ax C+ FS T1
Fig. 20.33
20.33Quiz 453
20.33 Answer
Cor T1 Ax T1
Ax C+ FS T1
Lipoblastoma (Chap. 4)
Child
Predominantly fatty mass
Multiple enhancing septa

Oncologic Imaging - ST Tumors

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Oncologic Imaging - ST Tumors

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Heung Sik Kang

Sung Hwan Hong

ISBN 978-981-287-717-8ISBN 978-981-287-718-5(eBook)

Library of Congress Control Number: 2017940416

Springer Science+Business Media Singapore 2017

Printed on acid-free paper

This Springer imprint is published by Springer Nature

To all the teachers and colleagues who have brought me to this

To my colleagues and family.

To my colleagues without whom I would not be here.

Diagnostic imaging of soft tissue tumors remains a challenge in musculoskel-

Seongnam, Korea, Republic of (South Korea) Heung Sik Kang

Part IGeneral Considerations for the Diagnosis of Soft

3.6 Illustrations: Staging ofSoft Tissue Sarcoma 29

Part IIWHO Classification of Soft Tissue Tumors and Radiologic

5.10 Calcifying Aponeurotic Fibroma 66

8 Pericytic (Perivascular) Tumors  131

11.3.1 Soft Tissue Chondroma 186

14 Undifferentiated/Unclassified Sarcoma  253

Part III Practical Pearls in Diagnosis of Soft Tissue Tumors

19.6 Neurofibromatosis Type 2 376

Part IV Drill and Practice

20 Image Interpretation Session 387

1.1 Clinical Consideration important for limiting the differential diagnosis

Springer Science+Business Media Singapore 2017 3

1.2 Imaging Modalities nonspecific (Hwang and Panicek 2009). US is in

1.4 Illustrations: Diagnostic Approach

1.4.1 Location ofSoft Tissue Tumors

Fig. 1.3 Elastofibroma dorsi. There are bilateral sym-

1.4.3 Radiographic Evaluation

1.4.4 Sonographic Evaluation

1.4.5 CT Evaluation

1.4.6 MR Imaging Evaluation

Garcia-Gomez JM, Vidal C, Marti-Bonmati L, Galant J,

2.1 Anatomic Compartments coracobrachialis, and brachioradialis muscles.

Springer Science+Business Media Singapore 2017 19

2.1.3 Lower Extremity 2.1.3.4 Extracompartmental Spaces

Gluteus max. Posterior

2.3.2 General Principles ofImage-Guided Percutaneous Biopsy

Fig. 2.6 Recommended biopsy needle path to avoid

Fig. 2.8 Lesions in the upper arm should be biopsied

ing surgery. Radiographics. 2007;27(1):189205.

Cancer staging is the process of determining how 3.1 Tumor (T-Staging)

Springer Science+Business Media Singapore 2017 27

AJCC prognostic stage groups

NewYork: Springer; 2016:507515

3.2 Nodes (N-Stage) sidered to occur through hematogenous and,

Table 3.2 FNCLCC grading system: Parameters for grading

Tumor necrosis No necrosis <50% tumor necrosis 50% tumor necrosis

Histologic grade (G) Total score

3.6.3 Stage III

References Leroux A, Jacquemier J, Duplay H, Sastre-Garau X,

Springer Science+Business Media Singapore 2017 39

At US, lipomas are hyperechoic masses, occa- 4.3 Lipomatosis ofNerve

4.9  typical Lipomatous Tumor/

Atypical lipomatous tumor or well-differentiated Dedifferentiated liposarcoma (DDLPS) is a sub-

tumor is characterized histologically by two myxoid liposarcomas often pathognomonically

4.12 Pleomorphic Liposarcoma (29%). The imaging appearance of pleomorphic

4.13.3 Lipomatosis ofNerve

4.13.6 Spindle Cell Lipoma

4.13.8 Lipoma Arborescens

4.13.9 Well-Differentiated Liposarcoma

4.13.10 Dedifferentiated Liposarcoma

4.13.11 Myxoid Liposarcoma

Seongnam, Korea, Republic of (South Korea) Heung Sik Kang

Part IGeneral Considerations for the Diagnosis of Soft

3.6 Illustrations: Staging ofSoft Tissue Sarcoma 29

Part IIWHO Classification of Soft Tissue Tumors and Radiologic

5.10 Calcifying Aponeurotic Fibroma 66

8 Pericytic (Perivascular) Tumors 131

11.3.1 Soft Tissue Chondroma 186

14 Undifferentiated/Unclassified Sarcoma 253

19.6 Neurofibromatosis Type 2 376

20 Image Interpretation Session 387

1.1 Clinical Consideration important for limiting the differential diagnosis

1.2 Imaging Modalities nonspecific (Hwang and Panicek 2009). US is in

1.4 Illustrations: Diagnostic Approach

1.4.1 Location ofSoft Tissue Tumors

1.4.3 Radiographic Evaluation

1.4.4 Sonographic Evaluation

1.4.5 CT Evaluation

1.4.6 MR Imaging Evaluation

2.1 Anatomic Compartments coracobrachialis, and brachioradialis muscles.

2.1.3 Lower Extremity 2.1.3.4 Extracompartmental Spaces

2.3.2 General Principles ofImage-Guided Percutaneous Biopsy

Cancer staging is the process of determining how 3.1 Tumor (T-Staging)

3.2 Nodes (N-Stage) sidered to occur through hematogenous and,

3.6.3 Stage III

At US, lipomas are hyperechoic masses, occa- 4.3 Lipomatosis ofNerve

4.9 typical Lipomatous Tumor/

4.12 Pleomorphic Liposarcoma (29%). The imaging appearance of pleomorphic

4.13.3 Lipomatosis ofNerve

4.13.6 Spindle Cell Lipoma

4.13.8 Lipoma Arborescens

4.13.9 Well-Differentiated Liposarcoma

4.13.10 Dedifferentiated Liposarcoma

4.13.11 Myxoid Liposarcoma

4.13.12 Pleomorphic Liposarcoma

Nodular fasciitis is a self-limiting reactive pro- 5.2 Proliferative Fasciitis

5.14 Solitary Fibrous Tumor

5.20.2 Proliferative Fasciitis

5.20.3 Proliferative Myositis

5.20.4 Myositis Ossificans

5.20.5 Elastofibroma Dorsi

5.20.6 Fibrous Hamartoma ofInfancy

5.20.7 Fibromatosis Colli

5.20.8 Fibroma ofTendon Sheath

5.20.9 Desmoplastic Fibroblastoma

5.20.10 Calcifying Aponeurotic

5.20.11 Palmar/Plantar Fibromatosis

5.20.12 Desmoid-Type Fibromatosis

5.20.14 Solitary Fibrous Tumor

5.20.17 Low-Grade Fibromyxoid

5.20.18 Low-Grade Myofibroblastic

5.20.19 Sclerosing Epithelioid

Thediffuse intra-articular lesions commonly 6.2 eep Benign Fibrous

6.4.2 Tenosynovial Giant Cell Tumor, Diffuse Type

6.4.3 Giant Cell Tumor ofSoft Tissue

6.4.4 Deep Benign Fibrous Histiocytoma

7.1 eiomyoma ofDeep Soft

9.1 Rhabdomyosarcoma occurs in adolescents and young adults. It more

9.2.2 Alveolar Rhabdomyosarcoma

9.2.3 Pleomorphic Rhabdomyosarcoma

9.2.4 Spindle Cell Rhabdomyosarcoma

10.3 Lymphangioma response. Surgical resection is the most effective

panied by lymphedema, appearing as a diffuse, 10.9 Illustrations: Vascular

10.9.2 Synovial Hemangioma

10.9.3 Intramuscular Angioma

10.9.4 Ossifying Intramuscular Angioma

10.9.5 Venous Hemangioma

10.9.6 Arteriovenous Malformation/Hemangioma

10.9.9 Kaposiform Hemangioendothelioma

10.9.10 Retiform Hemangioendothelioma

10.9.11 Kaposi Sarcoma

10.9.12 Epithelioid Hemangioendothelioma

10.9.13 Angiosarcoma ofSoft Tissue

11.3.2 Extraskeletal Osteosarcoma

detected in schwannomas that affect large, deep 12.2 Neurofibroma (Including

single imaging feature or combination of fea- 12.3 Perineurioma

12.6 Illustrations: Nerve Sheath Tumors