Malignant epithelioid angiomyolipoma of the submandibular gland: A case report.

An epithelioid angiomyolipoma (a perivascular epithelioid cell tumor) is a rare mesenchymal neoplasm with distinctive cellular morphology and nonspecific imaging appearances. Mostly reported perivascular epithelioid cell tumors (PEComas) are benign; however, rarely, PEComas can be malignant with pulmonary, hepatic, nodal, and osseous metastases. We present a case of a 40-year-old man with malignant right submandibular salivary gland PEComa, metastasized to the bones, lungs, and liver. We are going to discuss the diagnosis and management options of the rare disease of metastatic PEComa of the submandibular salivary gland.