Primary malignant PEComa of the mandible. Report of an unusual case

Malignant PEComa is a rare entity that usually origins at visceral, retroperitoneal and abdominopelvic sites. In the present paper, we describe an extremely unusual manifestation of malignant PEComa involving the mandible in a 48 years-old female patient focusing on the anatomopathological findings and differential diagnosis. The therapeutic management based on the head and neck tumor board indications is also discussed. Key words: Malignant PEComa, PEComa of the mandible, PEComa pathology, Oral cavity unusual neoplasm.