Epithelioid sarcoma overview: Difference between revisions
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==Overview== | ==Overview== | ||
Epithelioid sarcoma is a rare mesenchymal soft tissue [[tumor]] with an epithelioid pattern. It often occurs in the extremities of young people. | 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. | ||
Diagnosis is often delayed because of the unusual nature of the tumor. | |||
==Historical Perspective== | ==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. | 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. | ||
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Epithelioid sarcoma must be differentiated from synovial sarcoma, wart, ganglion cysts, rhabdomyosarcoma, and clear cell sarcoma. | Epithelioid sarcoma must be differentiated from synovial sarcoma, wart, ganglion cysts, rhabdomyosarcoma, and clear cell sarcoma. | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The epithelioid sarcoma is a very rare disease. The incidence of epithelioid sarcoma increases between ages of 10 and 39 and the average age of presentation was 27 years. Males are more commonly affected with epithelioid sarcoma than female. Epithelioid sarcoma of upper extremity usually affects individuals of the Caucasian race. | |||
==Risk Factors== | ==Risk Factors== | ||
The most potent risk factor in the development of epithelioid sarcoma is the presence of [[family history]] of epithelioid sarcoma. | |||
==Natural history, Complications and Prognosis== | ==Natural history, Complications and Prognosis== | ||
The symptoms of epithelioid sarcoma usually develop in the second decade of life, and start with symptoms such as mass. Epitheliod sarcoma has a tendency for lymph node metastasis. The most common sites of epithelioid m | |||
==History and Symptoms== | ==History and Symptoms== | ||
Revision as of 14:44, 19 February 2016
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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.
Epidemiology and Demographics
The epithelioid sarcoma is a very rare disease. The incidence of epithelioid sarcoma increases between ages of 10 and 39 and the average age of presentation was 27 years. Males are more commonly affected with epithelioid sarcoma than female. Epithelioid sarcoma of upper extremity usually affects individuals of the Caucasian race.
Risk Factors
The most potent risk factor in the development of epithelioid sarcoma is the presence of family history of epithelioid sarcoma.
Natural history, Complications and Prognosis
The symptoms of epithelioid sarcoma usually develop in the second decade of life, and start with symptoms such as mass. Epitheliod sarcoma has a tendency for lymph node metastasis. The most common sites of epithelioid m