Perivascular Epithelioid Cell Tumors (PEComa) is an extraordinarily rare mesenchymal neoplasm especially the malignant type originating from the lung. To date, only 8 cases of malignant or malignant potential pulmonary PEComa had been documented. | Malignant perivascular epithelioid cell tumor of the lung synchronous with a primary adenocarcinoma One case report and review of the literature