Congenital malignant teratoid neoplasm of the eye and orbit - A case report and review of the literature

Background: Medulloepithelioma is a tumor of the primitive medullary epithelium overlying the ciliary body. Most become evident early in life, and they may be malignant, although distant metastases are rare. The purpose of this report is to describe a unique case of congenital malignant teratoid neoplasm of the eye and orbit. Method: A patient with a congenital malignant teratoid tumor of the eye and orbit is described, and a detailed histopathologic study of the ocular findings with a review of the literature is presented. Results: Histopathologic study showed that the lesion was a malignant teratoid neoplasm with a large orbital extension. Several intracranial structural abnormalities were identified. Conclusion: The tumor described herein must be added to the differential diagnosis of congenital orbital masses. The clinician should be alert to the association of this lesion with complex intracranial abnormalities.