Perirenal perivascular epithelioid cell tumor (PEComa)

A 52-year-old woman, who is a carrier of MLH-1 mutation (HNPCC-type Lynch syndrome) with a history of colon adenocarcinoma, was diagnosed with a 26 mm lobulated contrast-capturing mass located caudally of the pancreas tail, anteromedial of the spleen and medial of the splenic colon angle. She underwent an exploratory laparotomy with resection of the tumor. Initially, this mass was presumed to be metastasis in a patient with a history of colon adenocarcinoma. However, after further histopathological and immunohistochemical examination, the mass appeared to be a rare PEComa. Only a few cases of a PEComa in this retroperitoneal perirenal location have been described.