Ameloblastic carcinoma of the mandible

BACKGROUND: Ameloblastic carcinoma is a rare, aggressive odontogenic neoplasm of the jaws in which the epithelial cells exhibit cytologic features of recognizable ameloblastoma and malignancy. Cases with metastasis have been infrequently reported. METHODS: A case of a 64-year-old white woman with mandibular ameloblastic carcinoma with documented distant metastasis is presented. The patient's presenting symptoms included facial asymmetry of the right jaw over 2 months and the development of moderate trismus. Clinical manifestations, pathology, treatment, and biologic behavior are discussed. The nomenclature and classification of odontogenic carcinomas are reviewed, including entities that should be considered in the differential diagnosis. RESULTS: The patient underwent surgical resection consisting of mandibulectomy, parotidectomy, and modified radical neck dissection followed by radiation to both necks and tumor bed. Postsurgically, the patient developed pulmonary metastasis at 11 months and expired with widespread metastatic disease at 28 months. CONCLUSIONS: This case demonstrated an unusual behavior pattern in that local recurrence and regional metastasis did not occur. Distant metastasis occurred despite apparent adequate control of the primary mandibular tumor. The ameloblastic carcinoma is a highly malignant neoplasm which requires aggressive therapy. Prognosis is poor. Further reporting of ameloblastic carcinoma is encouraged.