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

Abstract

Background: Perivasuclar epithelioid cell tumor (PEComa) is a rare group of mesenchymal tumors with unique histological and immunophenotypes. Retroperitoneal PEComa is extremely rare and almost benign lesion. Herein, we will present a case from China, focusing on histological and immunohistochemical characteristics of the tumor, follow-up and long-term outcome and the related literature review.