ABSTRACT
A case of ocular medulloepithelioma is reported and the authors review the literature concerning these rare tumors. This report concerns a ten-month-old girl in whom ophthalmic examination showed a large tumor arising in the ciliary body with such rapid progression that enucleation was necessary. Pathological findings were compatible with a malignant teratoid medulloepithelioma. According to preceding descriptions, slit lamp examination and ultrasonography are the best approach to the diagnosis. Medulloepithelioma is a tumor composed of poorly differentiated neuroepithelial cells and the predominant histological feature is a net-like pattern. Teratoid forms contain cartilage or brain tissue or bundles of skeletal muscle. The main criterion for malignancy is the presence of neuroblastic cells. Local excision is sufficient when the tumor is mall, otherwise enucleation or even exenteration may be necessary. The prognosis is generally good even when the tumor presents malignant features on pathological examination, provided treatment is instituted rapidly.
