Open AccessPerivascular epithelioid cell tumor of the gastrointestinal tract
Author(s) -
Tsung-Hao Liu,
CherWei Liang,
Li-Chun Lu
Publication year - 2016
Publication title -
journal of cancer research and practice
Language(s) - English
Resource type - Journals
eISSN - 2589-0425
pISSN - 2311-3006
DOI - 10.1016/j.jcrpr.2015.10.001
Subject(s) - medicine , gastrointestinal tract , pathology , perivascular epithelioid cell , epithelioid cell , immunohistochemistry
Perivascular epithelioid cell tumor (PEComa) is a rare subtype of soft tissue sarcoma that coexpresses melanocytic and smooth muscle markers. We report a case of malignant PEComa arising from the gastrointestinal tract. Definitive diagnosis of malignant PEComa is based on pathological examination and the standard treatment is complete surgical resection. Cytotoxic chemotherapy has limited efficacy in recurrent or metastatic malignant PEComa. Overactivation of the mammalian target of rapamycin (mTOR) pathway is observed in PEComa, and mTOR inhibitors have been recommended as an effective systemic treatment