Medulloepithelioma of the optic disc

SummaryA 6-year-old boy with a history of an amblyopic, occasionally red left eye was found to have a solid white mass overlying the optic disc on dilated ocular fundus examination. Transvitreal endoincisional biopsy of the mass yielded neoplastic tissue consistent with intraocular medulloepithelioma. The eye was removed subsequently because of concern that the tumor may invade the retrobulbar optic nerve. Histopathologic and immunohistochemical analysis of the tumor confirmed nonteratoid medulloepithelioma of the optic disc. The child has been followed up for over 36 months without any signs of orbital tumor recurrence or metastasis.