Perivascular epithelioid cell tumor of the oral mucosa

Perivascular epithelioid cell tumors (PEComas) are a family of tumors defined by the coexpression of melanocytic and muscle markers. Examples have been reported in many organs, soft tissues, and bone. Further expanding the list of locations, we report a case arising in the hard palate. Histologically, the tumor was composed of large elongated or epithelioid cells with granular cytoplasm. Immunohistochemically, tumor cells were positive for HMB-45, Melan A/MART-1, CD10, smooth muscle actin, desmin, and calponin. Ultrastructural examination revealed stage I melanosomes, thin filaments, and dense plaques. Recurrence has not been reported after 20 months. To our knowledge, this is the first detailed description of an intraoral PEComa.