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Diagnosis, Treatment, and Total Rehabilitation of a Secondary Type Ameloblastic Carcinoma.

Ameloblastic carcinoma (AC) is an uncommon malignant odontogenic tumor, with about 200 cases published in the literature. Here, we report a case of AC occurring in the maxilla - at the same anatomical location of an ameloblastoma 8 years earlier. A 23-year-old woman with maxillary swelling of 6-month duration was referred to our service. Extraoral examination revealed a swelling over the right side of the face, lifting her lip and nose. Intraorally, a firm, reddish, nodular swelling involving the right maxilla was observed. Computed tomography showed a hypodense image invading the right maxilla, maxillary sinus, and nasal fossa. An incisional biopsy was performed, followed by histopathologic examination and an immunohistochemical panel, which led to the diagnosis of AC. The lesion was treated with partial maxillectomy followed by immediate rehabilitation. Neither local recurrence of the tumor nor distant metastasis was observed during a 4-year follow-up. AC is a highly malignant lesion which requires aggressive therapy. Its diagnosis should be based on combined clinical, imaging, and pathological manifestations in order to improve diagnostic accuracy.