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Tumors 11
Joan C. Vilanova
Table 11.1 Changes and update relevant for radiologists in the 2013 WHO classification of tumors of the soft tissue
Tumor category Major changes
Adipocytic Mixed-type liposarcoma removed
Fibroblastic DFSP and giant cell fibroblastoma included for the first time
Myofibroblastic “Hemangiopericytoma” removed as a synonym for SFT
Recognition of nodular fasciitis and variants as true neoplasms
So-called fibrohistiocytic “Malignant fibrous histiocytoma” removed
Smooth muscle Angioleiomyoma reclassified as pericytic tumor
Pericytic Angioleiomyoma reclassified as a pericytic tumor
Myofibroma now classified as pericytic tumor
Skeletal muscle Spindle cell/sclerosing rhabdomyosarcoma classified together and
separated from others subtypes
Vascular Pseudomyogenic (epithelioid sarcoma-like)
Hemangioendothelioma added as a new entity
Gastrointestinal stromal GIST included in the volume on STT for the first time
Nerve sheath Peripheral nerve sheath tumors included volume on STT for the first time
New hybrid benign nerve sheath tumors included (schwannoma/
perineurioma)
Tumors of uncertain differentiation New tumors: acral fibromyxoma, hemosiderotic fibrolipomatous tumor
differentiation
Phosphaturic mesenchymal tumor
Atypical fibroxanthoma now included
PNET removed as synonym for Ewing’s sarcoma
Undifferentiated/other category Includes tumors that cannot be classified into any unclassified sarcoma
Abbreviations: WHO World Health Organization, DFSP dermatofibrosarcoma protuberans, SFT solitary fibrous tumor,
GIST gastrointestinal stromal tumor, STT soft tissue tumor, PNET primitive neuroectodermal tumor
Table 11.2 Group 1 – Adipocytic tumors Table 11.3 Group 2 – Fibroblastic/myofibroblastic
tumors
Benign
Lipoma Benign
Lipomatosis Nodular fasciitis (proliferative fasciitis/proliferative
myositis)
Lipomatosis of the nerve
Myositis ossificans
Lipoblastoma
Elastofibroma
Angiolipoma
Fibromatosis colli
Myolipoma of the soft tissue
Fibrous hamartoma of infancy
Chondroid lipoma
Juvenile hyaline fibromatosis
Spindle cell lipoma/pleomorphic lipoma
Inclusion body fibromatosis
Hibernoma
Fibroma of the tendon sheath
Intermediate (locally aggressive)
Desmoplastic fibroblastoma
Atypical lipomatous tumor/well-differentiated
liposarcoma Mammary-type myofibroblastoma
Malignant Calcifying fibrous tumor
Dedifferentiated liposarcoma Angiofibroma
Myxoid liposarcoma Angiomyofibroblastoma
Pleomorphic liposarcoma Gardner fibroma
Liposarcoma, not otherwise specified Calcifying fibrous tumor
Intermediate (locally aggressive)
Superficial fibromatosis (plantar/palmar)
coma chondroid lipoma usually shows calcifi- Desmoid-type fibromatosis
cations, which are best demonstrated by Lipofibromatosis
radiographs, CT, or US [3]. Giant cell fibroblastoma
Intermediate (rarely metastasizing)
Dermatofibrosarcoma protuberans
11.3 Fibroblastic/Myofibroblastic Solitary fibrous tumor
Tumors Inflammatory myofibroblastic tumor
Myofibroblastic sarcoma/atypical myxoinflammatory
Fibroblastic/myofibroblastic tumors represent a fibroblastic tumor
very large group of mesenchymal tumors, many Infantile fibrosarcoma
of which contain fibroblastic as well as myofi- Malignant
broblastic elements (Table 11.3). The current Adult fibrosarcoma
WHO classification recognizes that nodular fas- Myxofibrosarcoma
ciitis, proliferative fasciitis, and proliferative Low-grade fibromyxoid sarcoma
Sclerosing epithelioid fibrosarcoma
myositis (Fig. 11.1) are neoplastic [4], whereas
previously they were believed to be reactive
lesions. locally aggressive (intermediate) category
Other changes were the inclusion of the because it recurs in approximately 50 % of
closely related giant cell fibroblastoma and der- cases, but does not metastasize [6].
matofibrosarcoma protuberans (DFSP), for- Hemangiopericytoma has been removed from
merly included in volume on skin lesions. DFSP the classification of “extrapleural solitary
is categorized as a rarely metastasizing (inter- fibrous tumor” because it is well established that
mediate) tumor, although it should be noted that this outdated term included tumors that repre-
metastatic potential is gained only when a com- sented cellular examples of SFT [5]. Lipomatous
ponent of a fibrosarcomatous change is present hemangiopericytoma and giant cell angiofi-
[5]. Giant cell fibroblastoma is classified in the broma have also been included as SFT. The term
190 J.C. Vilanova
Table 11.5 Group 4 – Smooth muscle tumors Table 11.6 Group 5 – Pericytic (perivascular) tumors
Benign Benign
Leiomyoma of the deep soft tissue Glomus tumors (and variants)
Malignant Glomangiomatosis
Leiomyosarcoma (excluding the skin) Malignant glomus tumor
Myopericytoma
Myofibroma
a ngioleiomyoma (vascular leiomyoma) was real- Myofibromatosis
located to the category of pericytic (perivascular) Angioleiomyoma
tumors (group 5). There are no changes on the
deep leiomyosarcoma, although some tumors
Table 11.7 Group 6 – Skeletal muscle tumors
classified as undifferentiated pleomorphic sar-
coma closely resemble a subset of leiomyosar- Benign
coma, which may suggest the existence of Rhabdomyoma
Adult type
“dedifferentiated leiomyosarcoma.”
Fetal type
Genital type
Malignant
11.6 Pericytic (Perivascular)
Embryonal rhabdomyosarcoma
Tumors
Alveolar rhabdomyosarcoma
Pleomorphic rhabdomyosarcoma
The lesions in the pericytic/perivascular category
Spindle cell/sclerosing rhabdomyosarcoma
were first included in the 2002 edition. At the
same time, hemangiopericytoma was removed
from this group and listed as synonym for soli-
tary fibrous tumor (group 2). Although rare, the
most common tumor analyzed on imaging in this
group is glomus tumor [8], composed of cells
resembling the modified smooth muscle cells of
the normal glomus body. It has been acknowl-
edged that myofibroma/myofibromatosis repre-
sent morphological features along the spectrum
of myopericytic neoplasms (Table 11.6) instead
of fibroblastic/myofibroblastic tumors (group 2),
which were included in the past. Angioleiomyoma
(vascular leiomyoma) which typically presents as
a painful lesion in the distal extremities of
middle-aged adults has now been included in this
group [9, 10].
The prognosis of spindle cell rhabdomyosarcoma to be highly FDG-avid, and therefore PET-CT is
in adults is worse than in children, and recent useful for the detection of deep lesions.
insights into genetics of this tumor suggest that There are no changes on the classification of
there may be underlying genetic differences angiosarcoma and epithelioid hemangioendothe-
between pediatric and adult cases [12]. lioma (EHE) although new genetic data has
shown that multifocal EHE most likely arises as
a result of metastatic disease rather than repre-
11.8 Vascular Tumors senting synchronous primary tumors as previ-
ously thought.
Benign vascular tumors are very common and Immunohistochemical analysis can differenti-
most frequently occur in the skin. It is often dif- ate postradiation vascular proliferation from
ficult to determine whether they represent mal- angiosarcoma [15].
formations, true neoplasms, or reactive
processes [13]. Hemangiomas are classified his-
tologically as synovial, venous, arteriovenous, 11.9 Chondro-Osseous Tumors
mixed malformations, and intramuscular
(Table 11.8). “Pseudomyogenic (epithelioid sar- There are no changes on the chondro-osseous soft
coma-like) hemangioendothelioma” [14] has tissue tumors (Table 11.9). Myositis ossificans was
been added as a rarely metastasizing neoplasm. regarded as a (myo)fibroblastic lesion in the 2002
This tumor commonly arises in young adult version, and extraskeletal myxoid chondrosarcoma
males, often presents as multiple discontiguous (EMC) was also classified in the tumors of uncer-
nodules in different tissue planes of a limb tain differentiation, since it shows little evidence of
(Fig. 11.4), and may involve the skin as well as cartilaginous differentiation [2], despite the name.
deep soft tissues and the bone. This tumor tends A synonym for EMC is chordoid sarcoma.
Key Points
1. The WHO classification of soft tissue
tumors has been up-to-dated in 2013 for
the purpose of uniformity.
2. The new WHO classification has
included three new groups of tumors:
Fig. 11.8 The so-called synovial sarcoma of the lower gastrointestinal stromal tumors (GIST),
leg. Axial fat-suppressed T1-WI after contrast shows nerve sheath tumors, and undifferenti-
homogeneously enhancing lesion in the subcutaneous tis-
ated/unclassified sarcomas.
sue extending through the muscular fascia to deep com-
partment (arrow). As no nearby synovial tissue can be 3. Each group of tumors is divided in four
related to the mass in this location, the term “synovial” is categories regarding the biological
a misnomer for this lesion which is derived from undif- potential: benign, intermediate (locally
ferentiated mesenchymal stem cell
aggressive), intermediate (rarely metas-
tasizing), and malignant.
4. Radiologist should know the WHO
11.13 Undifferentiated/ classification to serve as a guide to work
Unclassified Sarcoma in a multidisciplinary committee with
clinicians, surgeons, and pathologists.
This new category of tumors, introduced for 5. Imaging (especially MRI) plays a pivotal
the first time in the 2013 classification, recog- role in the work-up of soft tissue tumors.
nizes the fact that a small, but significant, sub-
set of sarcomas cannot be classified into any
presently defined categories [23]. This group
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