Pigmented medulloepithelioma of the optic nerve: A challenging diagnostic entity

Because of its rarity, the diagnosis of optic nerve medulloepithelioma poses a real diagnostic challenge. Medulloepithelioma is a congenital tumor that derives from the primitive medullary epithelium present in the neural tube and the optic vesicle. Its classical location is the ciliary body. Cases...

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Bibliographic Details
Main Authors: Tiabi El Mehdi, Chariba Siham, Kamaoui Imane, Benajiba Noufissa, Bennani Amal
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-02-01
Series:Indian Journal of Pathology and Microbiology
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Online Access:https://journals.lww.com/10.4103/ijpm.ijpm_116_22
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Summary:Because of its rarity, the diagnosis of optic nerve medulloepithelioma poses a real diagnostic challenge. Medulloepithelioma is a congenital tumor that derives from the primitive medullary epithelium present in the neural tube and the optic vesicle. Its classical location is the ciliary body. Cases of retinal or optic nerve locations have been rarely reported in the literature. Only 11 cases have been published in the English literature. Herein, we report the case of a 2-year-old boy who underwent enucleation of the right eye for a presumed diagnosis of right-eye retinoblastoma, based on the presence of leukocoria on ophthalmological examination. Pathological examination showed an optic nerve medulloepithelioma. A review of the literature is also discussed in our work.
ISSN:0377-4929
0974-5130