Intraocular medulloepithelioma

Definition

Intraocular medulloepithelioma is a rare eye tumor characterized by a white, gray or yellow-colored cystic mass that arises from the primitive neuroectodermal, nonpigmented epithelium of the ciliary body, or occasionally from the optic nerve, optic disk, retina or iris. Typically it has a benign clinical course with good prognosis and generally presents with childhood onset of poor vision and pain, glaucoma, and/or cataract. Leukocoria, exotropia, exophthalmos, strabismus, epiphora, change in eye color, hyphema, and raised intraocular pressure are also remarkable manifestations.

Also known as intraocular medulloepithelioma, orbital medulloepithelioma — per MONDO

Also identified as

• NCIT C66806 per MONDO
• Orphanet 268139 per MONDO
• UMLS C1883694 per MONDO

Affected Organs