Retroperitoneal malignant perivascular epithelioid cell tumor: a rare case report and literature review

Abstract

Perivasuclar epithelioid cell tumor (PEComa) is a rare group of mesenchymal tumors with unique histological and immunophenotypes, which was first proposed by Zamboni in 1996 (1). PEComa can be occured in any ages, mostly in the middle-age. The lesions can involve in various parts of bodies, and uterus is the most common organ (2,3). Retroperitoneal PEComas are extremely rarely seen and almost all benign lesions (2,3). Herein, we present a case of malignant retroperitoneal PEComa with literature review to draw attention to this disease. Based on a literature review in PubMed and Web of Science with the terms of (“malignant perivascular epithelioid cell tumor” or “malignant PEComa”) and (“Post-peritoneal” or “retroperitoneal”), similar report of retroperitoneal malignant PEComa in this paper was rare reported before, especially the one from China (4,5). Compared with former cases, we focused on histological and immunohistochemical Case Report