Perirenal Perivascular Epithelioid Cell Tumor (PEcoma) with Pulmonary Micro Invasive Adenocarcinoma: A Case Report and Literature Review

Zhaoxun Li,1,* Nan Hu,2,* Yan Liao,1 Jingmei Liu1 1Department of Gastroenterology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, Hubei, People’s Republic of China; 2Department of Neurology and Laboratory of Clinical Genetics, Peking Union Medical College Hospital, Beijing, 100730, People’s Republic of China*These authors contributed equally to this workCorrespondence: Jingmei LiuDepartment of Gastroenterology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, People’s Republic of ChinaTel/Fax +86 2783665011Email LJM890202@163.comAbstract: Perivascular epithelioid cell tumor (PEComa) is a rare type of mesenchymal neoplasm, which occurs most commonly in uterus and gastrointestinal tract. PEComa with perirenal manifestation is an extremely rare entity. To the best of our knowledge, only four cases have been reported up to now. In this case, we reported a patient with both a pulmonary mass and a perirenal mass. Two resections were performed successively and postoperative pathology suggested pulmonary micro invasive adenocarcinoma (MIA) and perirenal PEComa. This is the first case of perirenal PEComa with pulmonary MIA. Combining the present case and prior literature, we summarized the crucial role of immunohistochemistry in the diagnosis and consider that complete operation might be conducive to patients with perirenal PEComa that presents a benign phenotype, regardless of complications with other tumors.Keywords: perivascular epithelioid cell tumor, pulmonary micro invasive adenocarcinoma, case repor