Pigmented malignant teratoid medulloepithelioma of ciliary body – An extremely rare intraocular tumor in adult

Malignant teratoid medulloepithelioma of ciliary body is an extremely rare tumor usually occurring in children younger than 5 years of age and is usually amelanotic. Here, we report a very rare case of pigmented malignant teratoid medulloepithelioma in a 20- year old male patient who presented initially with pain, redness and proptosis of right eyeball. The right eyeball was enucleated. A histopathological diagnosis of orbital teratoma was made and no further treatment was given. Four months later, the patient developed another swelling in the same area. A review of the previous histopathology slides revealed a pigmented malignant teratoid medulloepithelioma. No nerve involvement was seen. MRI did not show any intracranial extension. The mass along with rest of the orbital tissue was removed. No adjuvant chemotherapy or radiotherapy was given due to the lack of intracranial extension. The patient was symptom free at 2-year follow-up and the repeat MRI also showed no evidence of recurrence.