Ocular medulloepithelioma with teratoid features

Hanood Abu Rass, Cyrine Souki, Hussam Haddad


To report a rare case of intraocular malignant medulloepithelioma with teratoid features.
A 7 year old female patient presented with left intraocular mass. She underwent enucleation of her left eye with a preoperative diagnosis of intraocular retinoblastoma. Histopathology revealed this unusual tumor. Analysis of the clinical, histopathological and imaging characteristics of the tumor was performed.
Medulloepithelioma is a rare primitive tumor that has unique morphologic features recapitulating the medullary epithelial structuring of the primitive neural tube. In our case, the tumor is being further rare by manifesting divergent differentiation and maturation “teratoid features”.
Medulloepthelioma is a highly aggressive neoplasm that typically arises in the cerebrum of young children but also reported rarely the eye globe. Discussion of this rare challenging diagnosis, with emphasis on the distinctive histopathological characteristics and differential diagnosis is done.

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