Malignant PEComa involving the mandible: report of a unique case

We report a unique case of a malignant perivascular epithelioid cell neoplasm (PEComa) presenting as a slow-growing mandibular lesion in a 77-year-old Caucasian female. Primary osseous involvement by PEComas is rare. This is the first reported case of a malignant PEComa arising within the jaw. The patient is currently free of disease 2 years after treatment.