Maxillary Ameloblastoma with Orbital Involvement: An Institutional Experience and Literature Review.

Purpose: To describe 8 patients with orbital involvement by ameloblastoma and to review the literature on this topic.

Methods: The electronic medical records and pathology databases of the Hospital of the University of Pennsylvania were searched to identify all patients with histopathologically confirmed ameloblastoma diagnosed between 1990 and 2015. PubMed database was searched for all well-documented cases of maxillary ameloblastoma and ameloblastic carcinoma ex-ameloblastoma with orbital involvement published in the English literature. The information collected on the compiled 23 patients included age, sex, clinical presentation, imaging findings, management, tumor histopathologic features, and follow up.

Results: Review of medical records identified 8 patients with orbital involvement by ameloblastoma. Literature search yielded 15 patients with well-documented orbital involvement by ameloblastoma. Most tumors occurred in men (19 of 23, M:F = 4-5:1) with an average age of 56 years. The overall rates of recurrence, visual compromise, death, and confirmed disease-related mortality were 70% (16/23), 26% (6/23), 39% (9/23), and 22% (5/23), respectively. The initial surgical approach correlated with prognosis. The rates of recurrence, orbital exenteration, and mortality in the cohort managed with conservative surgery or partial maxillectomy were 57% (8/14), 29% (4/14), and 50% (7/14), respectively. In contrast, the patients initially managed with a radical resection had substantially lower frequencies of tumor recurrence (2/7, 29%), exenteration (1/7, 14%), and death (1/7, 14%). Malignant transformation to ameloblastic carcinoma occurred in the setting of recurrent disease in 3 patients and in 1 patient with prolonged duration of symptoms, suggestive of a long-standing tumor.

Conclusions: Maxillary ameloblastoma can rarely involve the orbit, leading to significant ocular morbidity and occasional mortality. Prompt radical resection of the tumor has the potential to decrease the likelihood of recurrence and visual compromise, and can improve survival.