Abstract
PURPOSE: Ameloblastoma is a non-encapsulated and slow-growing tumor with high recurrence rate. Orbital involvement by this neoplasm is an extremely rare entity. In this study, we present a systematic review on this situation along with clinical and paraclinical features of a case. METHODS: An electronic search was conducted on major medical sources. Data of the cases in the literature in addition to our own case were extracted, summarized, and statistically analyzed. RESULTS: A total of 36 other cases from 20 relevant studies were also reviewed. Review topics included epidemiology, clinical presentation, pathologic features, differential diagnosis, imaging, treatment, and prognosis. We provided a five-year history of a 50-year-old man with orbital/skull base invasion of plexiform maxillary ameloblastoma. CONCLUSIONS: Maxillary ameloblastoma is a locally aggressive neoplasm, and physicians must be alert to the biologic behavior of this tumor to detect any invasion to critical structures such as orbit and cranium. Orbital ameloblastoma causes significant morbidity and mortality. We advocate meticulous patient follow-up with regular clinical examinations and paraclinical work-up for timely detection of any invasion or recurrence. The best must be done to avoid extensions by aggressive removal of maxillary ameloblastoma.