Epithelioid sarcoma

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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

REFERENCES

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Chbani L, Guillou L, Terrier P, Decouvelaere AV, Grégoire F, Terrier-Lacombe MJ, Ranchère D, Robin YM, Collin F, Fréneaux P, Coindre JM: Epithelioid sarcoma: a clinicopathologic and immunohistochemical analysis of 106 cases from the French sarcoma group. Am J Clin Pathol 131:222-7,2009.

Enzinger FM: Epithelioid sarcoma: a sarcoma simjlating a granuloma or a carcinoma. Cancer 26:1029-41,1970.
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Eyden B, Wang G, Yao L: Epithelioid sarcoma: a case report with ultrastructural confirmation of myofibroblastic differentiation based on fibronexus junctions. Ultrastruct Pathol 33:61-6,2009.

Flucke U, Slootweg PJ, Mentzel T, Pauwels P, Husebos TJ: Re: Infrequent SMARCB1/INI1 gene alteration in epithelioid sarcoma: a useful tool in distinguishing epithelioid sarcoma from malignant rhabdoid tumor: Direct evidence of mutational inactivation of SMARCB1/INI1 in epithelioid sarcoma. Hum Pathol 40:1361-2,2009.

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Kim JH, Choi YS, Lee TS: A case of epithelioid sarcoma arising in the vulva. J Gynecol Oncol 19:202-4,2008.

Kohashi K, Izumi T, Oda Y, Yamamoto H, Tamiya S, Taguchi T, Iwamoto Y, Hasegawa T, Tsuneyoshi M: Infrequent SMARCB1/INI1 gene alteration in epithelioid sarcoma: a useful tool in distinguishing epithelioid sarcoma from malignant rhabdoid tumor. Hum Pathol 40:349-55,2009.
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Steib J-P, Pierchon F, Farcy JP, Lang G, Christmann D, Gnassia J-P: Epithelioid sarcoma of the spine: a case report. Spine 21:634-8,1996.
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von Hochstetter AR: Epithelioid sarcoma presenting as a chronic synovitis and mistaken for osteosarcoma. Skeletal Radiol 24:636-8,1995.
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