Abstract

Ameloblastic carcinoma (AC) is a rare and aggressive malignant epithelial odontogenic tumor making up less than 1% of malignant head and neck tumors. The majority of cases occur in the mandible with a minority occurring in the maxilla. Most occur de novo, while rare cases of AC have resulted from transformation from ameloblastoma. Here, we present a case in which a 30-year-old man presented with proptosis and a recurrent right temporal mass, which had been previously diagnosed as ameloblastoma on surgical pathology. CT findings demonstrated local invasion, and he was subsequently taken to the operating room for right craniotomy, infratemporal and middle cranial fossa tumor resection, and right modified radical neck dissection with reconstruction. Final pathology, which included areas of early focal necrosis, loss of peripheral palisading, and hyperchromatism, confirmed the diagnosis of ameloblastoma with transformation to AC. We further discuss radiologic and histopathological signs of this rare tumor, as well as recommended treatment modalities.

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