SOFT TISSUE

TUMORS
 EPIDEMIOLOGI
SOFT TISSUE : NON EPITHELIAL TISSUES,
EXCEPT : BONE, CARTILAGINEOUS,
BRAIN & MENINGES,
HEMATOPOETIC CELL,
LYMPHOID

SOFT TISSUE TUMORS : - BENIGN

- MALIGNANT

THE RATIO OF BENIGN TO MALIGNANT TUMORS > 100 : 1

50% OF PATIENTS SOFT TISSUE SARCOMA = DIE

10.000 NEW CASES OF SOFT TISSUE SARCOMA IN US / YEAR

ETIOLOGY AND GENETICS

MOLECULAR ABNORMALITY

CITOGENETIC ABNORMALITIES
 DISTRIBUTION
IT CAN OCCUR IN ANY SITE THROUGHOUT THE BODY

UPPER EXTREMITY : 13%

LOWER EXTREMITY : 30%
TRUNK : 10%
RETROPERITONEAL /
INTRA ABDOMINAL : 15%
VISCERAL : 19%
OTHER : 13%
HISTOLOGIC CLASSIFICATION OF SOFT
TISSUE TUMORS

FIBROUS TUMORS
FIBROHISTIOCYTIC TUMORS
LIPOMATOUS TUMORS
SMOOTH MUSCLE TUMORS
SKELETAL MUSCLE TUMORS
TUMORS OF BLOOD AND LYMPH VESSEL
PERIVASCULAR TUMORS
SYNOVIAL TUMORS
NEURAL TUMORS
PARAGANGLIONIC TUMORS
EXTRASKELETAL CARTILAGINOUS AND OSSEUS TUMORS
PLURIPOTENTIAL MESENCHYMAL TUMORS
MISCELLANOUS TUMORS
UNCLASSIFIED TUMOR
 FIBROUS TUMORS

Benign Tumors Superficial fibromatoses

Nodular fasciitis
Proliferative fasciitis & miositis
Atypical decubital fibroplasia
Fibroma
Keloid
Elastofibroma
Calcifying aponeurotic fibroma
Fibrous hamartoma of infancy
Fibromatosis coli
Infantile digital fibromatosis
Myofibromatosis
Juvenile hyaline fibromatosis
Calcifying fibrous pseudotumor
Fibromatoses
Dupuytrens contracture
Peyronies fibromatosis
Knuckle pads
Deep fibromatoses
Intermediate Tumors
Solitary fibrous tumors
Inflammatory myofibroblastic tu/
Cong/ or infantile fibrosarcoma
Malignant Tumors
Fibrosarcoma
Adult fibrosarcoma
Inflammatory fibrosarcoma
 TUMORS OF FIBROUS ORIGIN

FIBROSARCOMA
2 MAIN GROUPS : - THE ADULT TYPE
- INFANTILE TYPE
VERY UNCOMMON
ADULT FIBROSARCOMA : - 40 60 YEARS
- PAINFULL
- DEEP SEAT MASS
INFANTILE : 2 YEARS & OFTEN CONGENITAL
RECURREN AFTER EXCISION
HEMATOGEN METASTATIC PULMO
MORFOLOGY :
- SOLITER
- INFILTRATIF OR SIRCUMSCRIPTA
 TUMORS OF FIBROUS ORIGIN
FIBROSARCOMA

- CONSIST OF : ELONGATED FIBROBLAST-LIKE CELLS

ARRANGED IN A UNIFORM, HEARING BONE
PATTERN
- NUCLEUS ATYPIC & MITOTIC ACTIVITY
 FIBROHISTIOCYTIC TUMORS

Benign Tumors Malignant Tumors

Fibrous histiocytoma
Dermatofibroma
Deep fibrous histiocytoma
Juvenile xanthogranuloma
Reticulohistiocytoma
Xanthoma
Malignant fibrous histiocytoma
Storyform-pleomorphic
Myxoid (myxofibrosarcoma)
Giant cell
Inflammatory

Intermediate Tumors
Atypical fibroxanthoma
Dermatofibrosarcoma protuberans
Giant cell fibroblastoma
Flexiform fibrohistiocytic tumor
Angiomatoid fibrous histiocytoma
 FIBROHISTIOSYTIC TUMORS

MALIGNANT FIBROUS HISTIOCYTOMA

MFH FIRST INTRODUCED IN 1963

MALIGNANT SOFT TISSUE TUMOR WITH A FIBROHISTIOCYTIC
APPEARANCE
50 -70 YEARS
PAINLESS MASS
THE MOST COMMON SITE IS THE LOWER EXTREMITY;
FOLLOWED BY THE UPPER EXTREMITY & THE
RETROPERITONEUM
MORFOLOGY :
- GRAY-WHITE
- ENCAPSULATED
- INFILTRATIF
 FIBROHISTIOSYTIC TUMORS
MALIGNANT FIBROUS HISTIOCYTOMA

- SUB TYPE :
1. STORIFORM PLEOMORFIC : MOST COMMON
2. ANGIOMATOID : VASCULAR & TENDENCY YOUNGER
ADULT
3. INFLAMMATORIC
4. MIXOID
 LIPOMATOUS TUMORS
Benign Tumors Intermediate Tumors
Lipoma Atypical lipoma
Cutaneous lipoma
Deep Lipoma Malignant Tumors
Multiple lipomas Liposarcoma
Angiolipoma
Spindle cell or pleomorphic
lipoma
Miolipoma
Angiomyolipoma
Chondroid lipoma
Hibernoma
Lipoblastoma or
lipoblastomatosis
Lipomatosis
 LIPOMATOUS TUMORS

LIPOSARCOMA

INCIDENCE BETWEEN AGES 50 65

20% OF ALL SOFT TISSUE SARCOMA IN ADULT
OCCUR ANYWHERE IN THE BODY, THE MOST COMMON
ARE THE THIGH & THE RETROPERITONEUM
3 PRINCIPAL FORMS :
- WD OR DEDIFFERENTIATED
- MIXOID OR ROUND CELL
- PLEOMORPHIC
 SMOOTH MUSCLE TUMORS

Benign Tumors Malignant Tumors

Leiomyoma Leiomyosarcoma
Angiomyoma (vascular Epithelioid leiomyosarcoma
leiomyoma)
Epithelioid leiomyoma
Intravenous
leiomyomatosis
Leiomyomatosis
peritonealis diseminata
 SMOOTH MUSCLE TUMORS

LEIOMYOSARCOMA
MALIGNANT TUMOR
ANY LOCATION, MORE THAN HALF : RETROPERITONEAL OR
INTRAABDOMINAL SITES
CUTANEOUS LEIOMYOSARCOMAS : SMALL SOLITARY
EXTREMITY NODULES
RARE : ARISE IN LARGE VASCULAR STRUCTURE WITH
SYMPTOMS OF OBSTRUCTION TO THE NORMAL FLOW OF BLOOD
MORFOLOGY :
- TYPICAL CELL IS ELONGATED & HAS AN ABUNDANT CYTOPLASM
- MULTINUCLEATED GIANT CELLS

PROGNOSIS : MITOTIC ACTIVITY, LOCATION & SIZE

 SKELETAL MUSCLE TUMORS

Benign Tumors Malignant Tumors

Adult rhabdomyoma Rhabdomyosarcoma
Genital rhabdomyoma Rhabdomyosarcoma with
Fetal rhabdomyoma ganglionic diferentiation
Intermediate (cellular)-
rhabdomyoma
 SKELETAL MUSCLE TUMORS

RHABDOMYOSARCOMA

MALIGNANT TUMORS SHOWING SKELETAL DIFERENTIATION

REPRESENT THE LARGES SUBSET OF SOFT TISSUE
SARCOMAS IN INFANT AND CHILDREN
AGE : INFANT, CHILDREN & ADOLESCENT
AGE IS AN IMPORTANT PROGNOSTIC FACTOR FOR SURVIVAL
WORSE OUTCOME IN OLDER PATIENTS
HISTOLOGIC VARIAN :
- EMBRYONAL RHABDOMYOSARCOMA : MOST COMMON
- ALVEOLAR RHABDOMYOSARCOMA
- PLEOMORFIC RHABDOMYOSARCOMA
TUMORS OF BLOOD AND LYMPH VESSELS

Benign Tumors
Hemangioma

Malignant Tumors
Kaposis sarcoma
 TUMORS OF BLOOD AND LYMPH VESSELS

KAPOSIS SARCOMA

UNUSUAL VASCULAR SARCOMA

OCCUR IN THE SKIN
ELDERLY MEN
SPREAD TO THE LUNGS & GASTROINTESTINAL TRACT
RECEIVING IMMUNOSUPRESSANT THERAPY
EPIDEMIC : HIV
 SYNOVIAL TUMORS

Benign Tumors Malignant Tumors

Tenosynovial Giant Malignant giant cell
cell tumor tumor of tendon sheat
 CLINICAL STAGING

T PRIMARY TUMOR
T0 NO EVIDENCE OF PRIMARY TUMOR
T1 TUMOR < 5 CM IN GREATEST DIMENSION
T1a SUPERFICIAL TUMOR
T1b DEEP TUMOR
T2 TUMOR > 5 CM IN GREATEST DIMENSION
T2a SUPERFICIAL TUMOR
T2b DEEP TUMOR
N REGIONAL LYMPH NODES
N0 NO REGIONAL LYMPH NODES METASTASIS
N1 REGIONAL LYMPH NODES METASTASIS
M DISTANT METASTASIS
M0 NO DISTANT METASTASIS
M1 DISTANT METASTASIS