Perivascular epithelioid cell (PEC) tumors are rare tumors of mesenchymal origin and can affect many anatomic regions. Although these tumors are usually benign, malignant variants exist. We document for the first time a malignant PEComa arising from the pleura of a 43-year-old Malay woman. The pathology of the tumor as well as differential diagnoses is discussed.
Keywords: PEComa; differential diagnoses; malignant; pleura.