Perivascular epithelioid cell tumor of the gastrointestinal tract

Autor: Tsung-Hao Liu, Cher-Wei Liang, Li-Chun Lu
Jazyk: angličtina
Rok vydání: 2016
Předmět:
Zdroj: Journal of Cancer Research and Practice, Vol 3, Iss 1, Pp 14-18 (2016)
Druh dokumentu: article
ISSN: 2311-3006
DOI: 10.1016/j.jcrpr.2015.10.001
Popis: 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.
Databáze: Directory of Open Access Journals