DEFINITION AND PATHOGENESIS
- A slow, relentless, biphasic malignancy relatively common to the UE, consisting of epithelioid and spindle cells, with LR and metastases in young adults
IMPORTANCE
- Can be confused with an ulcerating skin lesion such as squamous cell carcinoma and benign granulomatous lesions, and inappropriately treated or even misdiagnosed as a draining abscess or "infected wart"
- Most common soft tissue sarcoma of the UE and the hand (some reports state synovial sarcoma as the most common in the hand)
6% of soft-tissue sarcomas
CLINICAL FEATURES
- Most patients 12-76 (average 39) years of age
- Types
- Conventional
- >50% occur on the fingers, hands, and wrists
- Has been reported within the knee presenting as chronic monoarthritis
- Proximal
- Rare on trunk, head and neck (except scalp)
- Has been reported in the lumbar spine
- Has been reported occuring in the vulva
- Usually not painful
- Multiple procedures for "recurrent growths" or "inflammatory granulomas"
- Up to 50% have incorrect "benign" dxs followed for an average of 35 months before correct dx made
- Woody but firm, painless lump which becomes ulcerated within a week
- M:F = 1.5:1
RADIOLOGIC FEATURES
- Occasionally (10-28% reported) with speckled calcifications ? cortical irregularities, rarely with frank ossification
- Infiltrative margins on MRI, seen on T2-weighted (low signal intensity) images best
- All have contrast enhancement
- 17% with a hemorrhagic component
GROSS PATHOLOGY
- Occur in the subcutaneous tissue, often ulcerated through the skin, firmly attached to underlying fascia, or deeper and poorly defined, spreading along fascial planes
- Usually 3-6cm in diameter, often multinodular, often <1cm
- Grayish ± focal areas of brown hemorrhage or necrosis (esp centrally)
HISTOLOGIC AND MOLECULAR FEATURES
- Eosinophilic, epithelioid-like poorly cohesive cells arranged in nodules with central areas of necrosis
- Biphasic
- Intracellular deposition of dense hyalin collagen is common
- Peripheral imflammatory cells along tumor margins
- Masson trichrome staining: cytoplasm deep red brown
- ± PAS for intracellular glycogen
- ± vascular invasion
- Dense intercellular reticulin
- Alcian blue: often + for mucin within the intercellular matrix
- Cytogenetic analysis: karyotype of 64-66 chromosomes
- Immunohistochemistry:
- + cytokeratin
- + vimentin
- + epithelial membrane antigen (EMA)
- Loss of INI1 expression is diagnostic
- EM: indented nuclei with chromatin along the margins, cytoplasmic filaments, intercellular junctions, lacks basal laminae
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Ulcerating squamous cell carcinoma
- Fibrosarcoma
- MFH
- Inflammatory processes
- Granuloma annulare
- Infectious granuloma
- Nodular fasciitis
- Rheumatoid nodule
- Granuomatous inflammation
- Synovial cell sarcoma
- Angiosarcoma
- Eithelioid hemangioendothelioma
- Malignant melanoma (+ S-100 and HMB-45)
- Sarcomatoid renal cell carcinoma
- Mesothelioma
- Extraskeletal osteosarcoma
- Rhabdomyosarcoma
DISEASE COURSE AND TREATMENT
- Recurrence (up to 77% reported), metastases (45%) to lymph nodes and lungs is the rule in tumors not treated with a wide resection or with delay in treatment
- Inconspicuously spreads along fascial planes leading to recurrence, even as late as 25 years after the initial excision
- Sentinel node evaluation/bx
- 10 year survival is 60% with wide or radical resection (<10% with marginal resection), 36% overall
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