INTRODUCTION Medulloepithelioma is a rare congenital tumor of the primitive medullary neuroepithelium. A significant proportion of patients with medulloepithelioma arising from the optic nerve die from intracranial spread or cerebral metastasis. Because it has no known distinct clinical features and because of its low frequency, this tumor presents within the first two to six years of life and ...

ntraocular medulloepithelioma is an uncommon embryogenic neoplasm of neuroepithelial origin that usually develops from the nonpigmented epithelium of the ciliary body, retina, and optic nerve.1 Clinically, medulloepithelioma usually appears as a tumor of the ciliary body; only rarely does it arise in the optic nerve and retina. It frequently occurs on the ciliary body of a young child as a fles...

Medulloepithelioma is a rare congenital tumor of the ciliary body and iris. The current classification and grading of medulloepithelioma shows inconsistencies and does not reflect clinical behavior. This position paper discusses the backgrounds of the current classification and intends to initiate a discussion on an alternative classification and grading scheme.

An 18-month-old Standardbred filly had a large intraocular tumor involving the optic nerve. The tumor was a malignant medulloepithelioma, a rare intraocular neoplasm derived from the primitive medullary epithelium. By light microscopy the tumor had cords and lobules of primitive neuroepithelial cells that formed clefts and true rosettes. Electron microscopy of the rosettes showed a girdle of zo...

A 7-year-old boy presented with angle-closure glaucoma, initially presumed to be idiopathic. A ciliary body mass was later detected on MR imaging, suggestive of medulloepithelioma but pathologically proved to be diffuse infiltrating retinoblastoma. We discuss the patient management and review the literature, with emphasis on the role of CT and MR imaging in evaluating pediatric angle-closure gl...

Intraocular medulloepithelioma is a rare embryonal neoplasm that occurs predominantly in children and arises from the primitive medullary epithelium of the optic cup. Medulloepitheliomas that contain heterotopic elements or tissues such as brain, cartilage, or rhabdomyoblasts are classified as teratoid, and tumors lacking these elements are classified as nonteratoid medulloepitheliomas. Both no...

The case is described of a benign medulloepithelioma in a child which originally was apparently confined to the iris.

A unique teratoid medulloepithelioma showing a conspicuous vascular component with orbital involvement is described.