Abstract
Medulloepithelioma (MEP) arises from the primitive neuroepithelium of the ciliary body of the eye, occurs in the first decade of life, and may be a manifestation of the DICER1 syndrome. It is often misdiagnosed with glaucoma or uveitis and became symptomatic with pain and visual loss when it is large enough to be seen through the pupil. MRI shows a mass composed by both a solid and a cystic component that histologically are represented by poorly differentiated neuroepithelium and cysts full of hyaluronic acid. Prognosis is excellent after enucleation (up to 95% 5-year survival), but remains poor when there is extraocular extension. Chemotherapy and radiotherapy may be used in selected cases.KeywordsMedulloepitheliomaOcular tumorDICER1 syndromeNeuroepitheliumVisual loss
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