• A slow, relentless, biphasic malignancy relatively common to the UE, consisting of epithelioid and spindle cells, with LR and metastases in young adults


  • 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


  • 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


  • 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


  • 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)


  • 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


  • 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


  • 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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