Perivascular Epithelioid Cell Neoplasm of the Lung: A Patient Case Report

Perivascular epithelioid cell tumors (PEComa) are a family of tumors characterized by epithelioid cells and are often found in perivascular spaces. Cases originating in the lungs are highly uncommon. We discuss the case of a pulmonary PEComa found in a 71-year-old male who had two instances of lung cancer 4 years prior. He had received a left lower lobectomy and a subsequent right upper lobectomy for separate primary Stage IA adenocarcinomas. His most recent surveillance imaging revealed a cystic lesion in the left upper lobe. A surgical course of treatment was recommended, and a wedge resection was performed. After immunohistochemical and genetic analysis, the lesion was characterized as a localized PEComa that was positive for Melan-A and Cathepsin-K on IHC, although negative for HMB-45. The current case contributes to the expanding histopathologic profile of these lesions.