A soft tissue neoplasm composed of perivascular epithelioid cells. Representative examples include angiomyolipoma, clear cell-sugar-tumor of the lung, and lymphangioleiomyomatosis. The disease is Monarch Disease Ontology identifier MONDO_0006359 (neoplasm with perivascular epithelioid cell differentiation). Also known as: PEComa, perivascular epithelioid cell neoplasm, perivascular epithelioid cell tumor, perivascular epithelioid cell tumour, tumor with perivascular epithelioid cell differentiation, tumour with perivascular epithelioid cell differentiation.