A 6-year-old girl had a blind, proptotic, and glaucomatous right eye. Preoperative diagnosis was glioma of the optic nerve. A malignant teratoid medulloepithelioma of the optic nerve was found, requiring orbital exenteration and removal of the intracanalicular and intracranial portions of the optic nerve. The child was alive and well with no evidence of metastasis after 18 months. To our knowledge, only two previous cases of this rare tumor have been observed.
Green WR, Iliff WJ, Trotter RR. Malignant Teratoid Medulloepithelioma of the Optic Nerve. Arch Ophthalmol. 1974;91(6):451-454. doi:10.1001/archopht.1974.03900060465007