Intraocular medulloepithelioma: an unusual and challenging entity in paediatric population

Authors

  • Uzma Imam Department of Paediatric Oncology, National Institute of Child Health, Karachi, Pakistan
  • Marya Hameed Department of Radiology, National Institute of Child Health, Karachi, Pakistan
  • Irma Anis Department of Paediatrics, Civil Hospital, Karachi, Pakistan
  • Israr Bhutto Department of Ophthalmology, Al Ibrahim Eye Hospital, Karachi, Pakistan
  • Syed Habib Ahmed Department of Paediatric Oncology, National Institute of Child Health, Karachi, Pakistan
  • Attiya Rizvi Department of Ophthalmology, Al Ibrahim Eye Hospital, Karachi, Pakistan
  • Zubair Ahmad Department of Histopathology, Aga Khan University Hospital, Karachi, Pakistan

DOI:

https://doi.org/10.47391/JPMA.8253

Keywords:

Pediatric, Medulloepithelioma, DICER1 associated tumors

Abstract

Intraocular medulloepithelioma is a rare, congenital tumour of the non-pigmented ciliary epithelium. It most frequently arises from the ciliary body but can also have its origin from the retina, iris and optic nerve. The age when lesion first appears is typically around 2-10 years. Nearly 50-60% of patients having this lesion may also have secondary features such as cataract and neovascular glaucoma. Those with extrascleral medulloepithelioma are at risk for metastasis. Systemic correlation of the tumour with pleuropulmonary blastoma/DICER1 gene is reported in the literature.

Here, we report a case of a 15 years old boy with one year history of right eye proptosis and painful red right eye along with decreased vision for one week. He was assessed and operated for cataract elsewhere three years back.

---Continue

Published

2023-11-28

How to Cite

Uzma Imam, Marya Hameed, Anis, I., Israr Bhutto, Syed Habib Ahmed, Attiya Rizvi, & Zubair Ahmad. (2023). Intraocular medulloepithelioma: an unusual and challenging entity in paediatric population. Journal of the Pakistan Medical Association, 73(12), 2469–2472. https://doi.org/10.47391/JPMA.8253

Issue

Section

Case Report

Most read articles by the same author(s)