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proximal-type epithelioid sarcoma
Tuesday 27 September 2005
Definition: Proximal-type epithelioid sarcoma is a recently described soft-tissue tumor that is distinguished from conventional-type epithelioid sarcoma by a far more aggressive clinical course, frequent location in the proximal anatomic regions, and variable rhabdoid morphology.
Because of their rarity and peculiar morphology, proximal-type epithelioid sarcomas frequently pose serious diagnostic dilemmas, being easily misdiagnosed as a variety of other malignant neoplasms.
Synopsis
- pelvic and perineal areas
- young to middle-aged adults
- proliferation of epithelioid-like cells with rhabdoid features in the absence of a granuloma-like pattern
- immunohistochemistry: vimentin+, cytokeratin+, epithelial membrane antigen+, CD34+
- differential diagnosis: tumors with rhabdoid features, extrarenal malignant rhabdoid tumor
Cytogenetics
translocations
chromosome arm 8q gain
- target genes: MYC with MYC gene copy increase
References
Proximal-type epithelioid sarcoma of the vulva: relationship to malignant extrarenal rhabdoid tumor. Tholpady A, Lonergan CL, Wick MR. Int J Gynecol Pathol. 2010 Nov;29(6):600-4. PMID: 20881848
Proximal-type epithelioid sarcoma of the maxilla with metastasis to the scalp: Cytologic findings of an unusual malignant tumor. Uschuplich V, Gadre SA, Debski RF, Sahoo S. Diagn Cytopathol. 2010 Oct;38(10):744-7. PMID: 20058307
Proximal-type epithelioid sarcoma in the groin presenting as a diagnostic dilemma. Mannan AA, Rifaat AA, Kahvic M, Kapila K, Mallik M, Grover VK, Bharati C, Perry A. Pathol Oncol Res. 2010 Jun;16(2):181-8. PMID: 19757197
Lualdi E, Modena P, Debiec-Rychter M, Pedeutour F, Teixeira MR, Facchinetti F, Dagrada GP, Pilotti S, Sozzi G. Molecular cytogenetic characterization of proximal-type epithelioid sarcoma. Genes Chromosomes Cancer. 2004 Nov;41(3):283-90. PMID: 15334553