[Intraocular medulloepithelioma - series of 10 cases and review of the literature]

Abstract

Intraocular medulloepithelioma is an extremely rare unilateral intraocular tumor arising from the nonpigmented ciliary epithelium. Medulloepitheliomas may be classified as benign and malignant and as teratoid and nonteratoid tumors. As a rule a long latency period occurs after first symptoms until the final diagnosis of a medulloepithelioma is made. Differential diagnosis includes in particular unilateral retinoblastoma. Intraocular medulloepithelioma may occur as masquerade-syndrome simulating uveitis. We present 10 patients with intraocular medulloepithelioma. In 7 of these patients the eye had to be enucleated. Metastasis did not occur, but epiretinal tumor seeding did occur in one patient. In one of the 3 not enucleated eyes, ruthenium-106 brachytherapy could salvage the tumor containing eye.