ASPL-TFE3 translocation in vulvovaginal alveolar soft part sarcoma
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Lippincott Williams and Wilkins
Abstract
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. © 2014 International Society of Gynecological Pathologists.
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Keywords
Alveolar soft part sarcoma, Aspl-tfe3 gene fusion, Tfe3 protein, Vagina, Vulva, Basic helix-loop-helix leucine zipper transcription factors, Female, Gene expression regulation, neoplastic, Humans, Oncogene proteins, fusion, Sarcoma, alveolar soft part, Translocation, genetic, Vaginal neoplasms, Vulvar neoplasms, Young adult, Membrane protein, Protein tfe3, Unclassified drug, Aspscr1 protein, human, Basic helix loop helix leucine zipper transcription factor, Oncoprotein, Tfe3 protein, human, Adult, Article, Aspl tfe3 gene, Cancer radiotherapy, Case report, Cesarean section, Computer assisted tomography, Fusion gene, Gene rearrangement, Gene translocation, Histopathology, Human, Human tissue, Immunohistochemistry, Local excision, Postoperative period, Priority journal, Protein expression, Third trimester pregnancy, Vulvovaginal alveolar soft part sarcoma, Gene expression regulation, Genetics, Pathology, Vagina tumor, Vulva tumor