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Updated: Apr 8, 2026

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Intraocular Ependymoma in a Child - Case report.

Yehonatan Weinberger1,2, Jonathan Weidenfeld2,3, Ido Didi Fabian2,4

  • 1Ophthalmology department, Rabin Medical Center, Petah-Tikva, Israel.

Retinal Cases & Brief Reports
|April 7, 2026
PubMed
Summary
This summary is machine-generated.

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This case report details a rare childhood intraocular ependymoma, initially misdiagnosed as medulloepithelioma. Diagnosis was confirmed via histology and molecular studies after treatment failure and enucleation.

Area of Science:

  • Ophthalmology
  • Pediatric Oncology
  • Neuropathology

Background:

  • Intraocular tumors in children are rare and often challenging to diagnose.
  • Ciliary body medulloepithelioma is a common intraocular tumor in childhood.
  • Accurate diagnosis is crucial for appropriate management and prognosis.

Purpose of the Study:

  • To report an extremely rare case of intraocular ependymoma in a child.
  • To highlight diagnostic challenges and the importance of comprehensive pathological analysis.
  • To emphasize the need to consider intraocular ependymoma in the differential diagnosis of pediatric intraocular tumors.

Main Methods:

  • Case report detailing clinical presentation, treatment course, and enucleation.
  • Histopathological examination including immunohistochemistry (GFAP, S100, EMA, L1CAM, Olig2).
Keywords:
EpendymomaIntraocular tumorPediatric ophthalmology

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  • Molecular genetic studies to identify specific fusion sequences (C11orf95-RELA).

    • Main Results:

    • A 5-year-old boy was initially diagnosed with ciliary body medulloepithelioma.
    • Treatment with plaque brachytherapy failed, leading to neovascular glaucoma and enucleation.
    • Histology revealed atypical cells in pseudorosettes, and immunohistochemistry/molecular studies confirmed intraocular ependymoma.

    Conclusions:

    • Intraocular ependymoma is an exceptionally rare diagnosis in pediatric ophthalmology.
    • Comprehensive histopathological and molecular analysis is essential for definitive diagnosis.
    • Intraocular ependymoma should be considered in the differential diagnosis of pediatric retinal and ciliary body tumors.