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Epithelioid sarcoma

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

Abbas O, Ghosn S, Chedraoul A, Kibbi AG: Giant fleshy mass over the left axilla: Epithelioid sarcoma (ES) (metastatic). Arch Dermatol 145:589-94,2009.

Andreou D, Tunn PU: Sentinel node biopsy in soft tissue sarcoma. Recent Results Cancer Res 179:25-36,2009.

Armah HB, Parwani AV: Epithelioid sarcoma. Arch Pathol Lab Med 133:814-9,2009.

Bos GD, Pritchard DJ, Reiman HM, : Epithelioid sarcoma. An analysis of 51 cases. J Bone Joint Surg 70A:862-70,1988.
Brassesco MS, Valera ET, Castro-Gamero AM, Moreno DA, Silveira TP, Mori BM, Engel EE, Scrideli CA, Tone LG: Cytogenetic findings in an epithelioid sarcoma with angiomatoid features. A case report. Genet Mol Res 8:1211-7,2009.

Burgos AM, Chavez JG, Sanchez JL, Sanchez NP: Epithelioid sarcoma: a diagnostic and surgical challenge. Dermatol Surg 35:687-91,2009.

Chase DR, Enzinger EM: Epithelioid sarcoma. Diagnosis, prognositic indicators and treatment. Am J Surg Pathol 9:241-63,1985.

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.
Epithelioid sarcoma: a cse report with ultrastructural confirmation of myofibroblastic differentiation based on fibronexus junctions. Ultrastruct Pathol 33

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.

Fujii H, Honoki K, Yajima H, Kido A, Kobata Y, Kaji D, Takakura Y: Epithelioid sarcoma of the forearm arising from perineural sheath of median nerve mimicking carpal tunnel syndrome. Sarcoma 2009:595391.

Hoshino M, Kawashima H, Ogose A, Kudo N, Ariizumi T, Hotta T, Umezu H, Hatano H, Morita T, Nishio J, Iwasaki H, Endo N: Serum CA125 expression as a tumor marker for diagnosis and monitoring the clinical course of epithelioid sarcoma. J Cancer Res Clin Oncol        2009.

Hurtado RM, McCarthy E, Frassica F, Holt PA: Intraarticular epithelioid sarcoma. Skeletal Radiol 27:453-6,1998.

Jawad MU, Extein J, Min ES, Scully SP: Prognostic factors for survival in patients with epithelioid sarcoma: 441 cases from the SEER database. Clin Orthop 467:2939-48,2009.

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.
Lo H, Kalisher L, Faix JD: Epithelioid sarcoma: radiologic and pathologic manifestations. AJR 128:1017-20,1977.
Maduekwe UN, Hornicek FJ, Springfield DS, Raskin KA, Harmon DC, Choy E, Rosenberg AE, Nielsen GP, DeLaney TF, Chen YL, Ott MJ, Yoon SS: Role of sentinel lymph node biopsy in the staging of synovial, epithelioid, and clear cell sarcomas. Ann Surg Oncol 16:1256-63,2009.

Mannan AA, Rifaat AA, Kahvic M, Kapila K, Mallik M, Grover VK, Bharati C, Perry AP Proximal-type epithelioid sarcoma in the groin presenting as a diagnositc dilemma. Pahtol Oncol Res               2009.

McGrory JE, Pritchard DJ, Arndt CAS, Nescimento AG, Remstein ED, Rowland CM: Nonrhabdomyosarcoma soft tissue sarcomas in children: The Mayo Clinic Experience. Clin Orthop 374:247-58,2000.

Nishimura Y, Yamaguchi Y, Tomita Y, Hamada K, Maeda A, Morita A, Katayama I: Epitheoid sarcoma on the foot masquerading as an intractable wound for > 18 years. Clin Exp Dematol            2009.

Orrock JM, Abbott JJ, Gibson LE, Folpe AL: INI1 and GLUT-1 expression in epithelioid sarcoma and its cutaneous neoplastic and nonneoplastic mimcs. Am J Dermatopathol 31:152-6,2009.
Prat J, Woodruff JM, Marcove RC: Epithelioid sarcoma: an analysis of 22 cases indicating the prognostic significance of vascular invasion and regional lymph node metastasis. Cancer 41:1472-87,1978.

Rai H, Odunsi K, Kesterson J, Morrison C, Chan A, Mhawech-Fauceglia P: Epithelioid sarcoma of the vulva in a 17-year-old pregnant woman. Appl Immunohistochem Mol Morphol 17:270-3,2009.
Romero JA, Kim EE, Moral IS: MR characteristics of epithelioid sarcoma. J Comput Assist Tomogr 18:929-31,1994.
Raoux D, Péoc'h M, Pedeutour F, Vaunois B, Decouvelaere AV, Folpe AL: Primary epithelioid sarcoma of bone: report of a unique case with immunohistochemical and fluorescent in situ hybridization confirmation of INI1 deletion. Am J Surg Pathol 33:954-8,2009.

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.
Ueda Y, Chijiwa H, Nakashima T: Epithelioid sarcoma of the neck: case report. J Laryngol Otol 31 Suppl 31:113-6,2009.

von Hochstetter AR: Epithelioid sarcoma presenting as a chronic synovitis and mistaken for osteosarcoma. Skeletal Radiol 24:636-8,1995.
Watabe A, Okuyama R, Hashimoto A, Hosaka M, Hatori M, Kariya Y, Watanabe M, Hashimoto H, Tagami H, Aiba S: Epithelioid sarcoma-like haemangioendothelioma: a case report. Acta Derm Venereol 89:208-9,2009.

Wilkins RM, Pritchard DJ, McLeod RA, Reiman HM: Epithelioid sarcoma (clinical conference). Orthopedics 8:421-3,1985.
Wilson SC, Cascio BM, Randaisi V, Levine E, Chuinard RG, Neitzschman HR: Slowly growing mass on the thumb of a 24-year-old woman. Clin Orthop 367:315-8,20-2,1999.

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