Primary ameloblastic carcinoma of the maxilla: A case report and literature review.

Ameloblastic carcinoma (AC) is a rare malignant odontogenic neoplasm that tends to occur in the mandible rather than in the maxilla. This malignancy is classified as a tumor that combines the morphological features of ameloblastoma and carcinoma, regardless of the presence or absence of metastasis. In addition, AC has been classified into two types, primary and secondary. The former develops and the latter develops by malignant transformation of a pre-existing benign ameloblastoma. The present study describes the case of a 22-year-old patient with primary AC of the maxilla. A review of the literature focusing on the clinical details, treatment results and histopathological and phenotypic information available for ameloblastic carcinoma of the maxilla from a 60-year period was also performed. As a result, it was found that primary AC is dominant in the maxilla and does not exhibit an aggressive phenotype compared with secondary AC. In addition, the presence of recurrence was found to correlate with mortality, indicating that early, aggressive and complete removal of the tumor is the best treatment for survival.