Neuro-ophthalmologic aspects of ameloblastoma

Ameloblastomas are histologically benign tumors derived from the odontogenic apparatus. Although these tumors are locally invasive, they rarely invade the paranasal sinuses, orbits, or intracranial cavity, and, thus, they rarely produce ophthalmologic signs and symptoms. In this report, we describe the neuro-ophthalmologic features of three patients with chronically aggressive ameloblastomas. Two of the patients developed a progressive and recurrent orbital apex and cavernous sinus syndromes. One of these patients is, to our knowledge, the first patient described with orbital and cavernous sinus involvement by an ameloblastoma initially arising in the mandible. The other is only the second case described with bilateral orbital involvement. The third patient in this series developed a trigeminal sensory neuropathy as the only sign of the tumor. Although ameloblastomas are benign, slowly growing tumors, they may, often over a long period of time, cause significant neuro-ophthalmologic and orbital manifestations that can only be partially ameliorated by surgery.