ASPL-TFE3 translocation in vulvovaginal alveolar soft part sarcoma.

Int J Gynecol Pathol

Departments of Pathology and Laboratory Medicine (M.N.J., G.S.Z.) Obstetrics and Gynecology (M.S.), American University of Beirut Medical Center, Beirut, Lebanon Department of Pathology (H.AL-A), Memorial Sloan-Kettering Cancer Center, New York, New York Department of Anatomic Pathology (F.W.A-K), Cleveland Clinic, Cleveland, Ohio.

Published: May 2014

Alveolar soft part sarcoma of the vulvovaginal region is limited to only 8 reported vaginal cases and 1 vulvar case in the English literature. The histogenesis of the tumor remains intriguing with postulates favoring a myogenic versus nonmyogenic origin. A reciprocal translocation for ASPL-TFE3 gene fusion, frequently detected in ~90% of cases, combined with TFE3 protein immunoexpression are highly sensitive and specific methods for diagnostic confirmation. The current report describes a unique case of vulvovaginal alveolar soft part sarcoma showing the classic morphologic features with documentation of TFE3 protein expression and the ASPL-TFE3 gene rearrangement. Furthermore, a brief review of the literature of vulvar and vaginal alveolar soft part sarcoma cases with the various treatment modalities is outlined.

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Source
http://dx.doi.org/10.1097/PGP.0b013e318290407cDOI Listing

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