Intraocular malignant teratoid medulloepithelioma in an adult: clinicopathological case report and review of the literature

Abstract

To report a case of intraocular medulloepithelioma, an embryonal tumour with extremely rare presentation in adults. The case of a 44-year-old man with intraocular malignant teratoid medulloepithelioma, primarily diagnosed as intraocular teratoma, is described and the literature on this subject is reviewed. The patient presented with progressive proptosis caused by a tumour in the left eyeball. He had a 28-year history of loss of vision in the left eye. Histopathological examination of the enucleated eye demonstrated an intraocular teratoma. No adjuvant treatment was given. Six months later the patient presented with massive progression in the left orbit and intracranial invasion. Cisplatin-based chemotherapy was administered, but discontinued after two cycles due to poor tolerance and lack of response. At subsequent pathology review, a final diagnosis of malignant teratoid medulloepithelioma was made. Salvage radiotherapy (60 Gy in 30 fractions) resulted in partial response of the intracranial lesion. However, the patient died 6 months later due to intracranial tumour progression. Medulloepithelioma should be considered in the differential diagnosis of intraocular tumours in adults, especially in the case of coexisting, long-standing ocular symptoms. In some cases this disease is very aggressive.