Epithelioid sarcoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Jesus Rosario Hernandez, M.D. [2]; Ammu Susheela, M.D. [3]
Overview
Epithelioid sarcoma is a rare mesenchymal soft tissue tumor with an epithelioid pattern. It often occurs in the extremities of young people.
Diagnosis is often delayed because of the unusual nature of the tumor.
Historical Perspective
Epithelioid sarcoma was first discovered by Dr. J. Laskowski, a polish doctor, in 1961 following the description of a clinical entity called sarcoma aponeuroticum due to involvement of aponeurosis. Enzinger coined the term epithelioid sarcoma in 1970.
Pathophysiology
Mutations in the SMARCB1 gene cause epithelioid sarcoma. On gross pathology, solid, multinodular mass, glistening gray tan appearance, and multiple areas of hemorrhage and necrosis are characteristic findings of epithelioid sarcoma. On microscopic histopathological analysis, central necrosis surrounded by bland, polygonal cells with eosinophilic cytoplasm and peripheral spindling, desmoplasia, focal calcification, or metaplastic ossification are characteristic findings of epithelioid sarcoma.
Causes
Mutations in the SMARCB1 gene cause epithelioid sarcoma.
Differentiating Epithelioid sarcoma from other Diseases
Epithelioid sarcoma must be differentiated from synovial sarcoma, wart, ganglion cysts, rhabdomyosarcoma, and clear cell sarcoma.