Odontogenic carcinoma differentiation into rhabdomyosarcoma: report of a rare case.

: To the authors' knowledge, this is the first reported case of an odontogenic carcinoma with documented skeletal muscle differentiation (rhabdomyosarcoma). The histology and clinical features of this aggressive odontogenic neoplasm are described. Within the English-language literature, only 2 cases are reported of an odontogenic tumor with muscle differentiation: a benign odontogenic tumor (ameloblastoma) with differentiation into a rhabdomyosarcoma and an odontogenic sarcoma with smooth-muscle differentiation. The general practitioner should be aware that odontogenic lesions may be malignant, even though this is extremely rare, and all tissue removed from the oral cavity should be submitted for biopsy.