RESUMEN
Ameloblastic carcinoma (AC) is an uncommon malignant odontogenic tumor that can be difficult to differentiate from ameloblastoma and can arise directly as an undifferentiated lesion or from a pre-existing benign lesion. The current study presents a novel case of primary maxillary AC and review the literature on AC of the maxilla. The review of the literature indicates that secondary tumors and posterior localization are associated with a higher tendency for recurrence and, often, multiple recurrences. Surgical therapy, eventually followed by radiotherapy, is the treatment modality most frequently applied, while the role of chemotherapy remains unclear. Several new cases of maxillary AC have been recently described in literature, making this pathology more frequent than previously considered; this is perhaps an indication of an increased diagnostic sensibility, rather than a real increase in the incidence of the disease itself.