Ophthalmic Knowledge for Residents: Pathology

Epithelium Neuroepithelial Primitive Optic Shows Cysts Medulloepithelioma Rare

Front

What is a medulloepithelioma?

Back

● Rare tumor of nonpigmented epithelium of the ciliary body ● Neuroepithelial cell derived from primitive medullary epithelium (inner layer of optic cup) ● Usually presents in children age 4-12 years old ● Can be benign or malignant ● Rarely causes secondary glaucoma, hyphema, ectopia lentis ● Pathology shows ribbons and cords of primitive round and oval cells, multiple layers of neuroepithelial tables lined by thin basement membrane; mucinous cysts and Flexner-Wintersteiner rosettes ● Can be seen in the retina and optic nerve ● B scan shows large cysts ● Treatment: observation or enucleation before orbital invasion (do not resect as it induces metastatic spread)

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