Question:medium
A child presenting with whitish pupillary reflex was treated with enucleation. Histopathology of the specimen showed Flexner-Wintersteiner rosettes. What is the diagnosis?
A child presenting with whitish pupillary reflex was treated with enucleation. Histopathology of the specimen showed Flexner-Wintersteiner rosettes. What is the diagnosis?
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Flexner-Wintersteiner rosettes are pathognomonic for the most common intraocular tumour of childhood.
Updated On: Jun 23, 2026
- Retinoblastoma
- Rhabdomyosarcoma
- Medulloblastoma
- Astrocytoma
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The Correct Option is A
Solution and Explanation
The triad to recognise: (1) child, (2) leukocoria (white pupillary reflex), (3) Flexner-Wintersteiner rosettes on histopathology.
This combination is pathognomonic of Retinoblastoma.
Histopathological features of retinoblastoma:
- Small hyperchromatic cells with a high nuclear-to-cytoplasmic ratio
- Large areas of necrosis with multifocal calcification
- Flexner-Wintersteiner rosettes: tumour cells arranged around a central lumen with a limiting membrane -- represent photoreceptor differentiation (poorly differentiated, <50%)
- Homer-Wright rosettes: cells around a central tangle of fibrils -- seen in well-differentiated (>50%) retinoblastoma
Genetics: inactivation of RB1 tumour suppressor gene (chromosome 13q14) -- two-hit hypothesis (Knudson).
Rhabdomyosarcoma, medulloblastoma, and astrocytoma do not show Flexner-Wintersteiner rosettes. \[\boxed{\text{Retinoblastoma}}\]
This combination is pathognomonic of Retinoblastoma.
Histopathological features of retinoblastoma:
- Small hyperchromatic cells with a high nuclear-to-cytoplasmic ratio
- Large areas of necrosis with multifocal calcification
- Flexner-Wintersteiner rosettes: tumour cells arranged around a central lumen with a limiting membrane -- represent photoreceptor differentiation (poorly differentiated, <50%)
- Homer-Wright rosettes: cells around a central tangle of fibrils -- seen in well-differentiated (>50%) retinoblastoma
Genetics: inactivation of RB1 tumour suppressor gene (chromosome 13q14) -- two-hit hypothesis (Knudson).
Rhabdomyosarcoma, medulloblastoma, and astrocytoma do not show Flexner-Wintersteiner rosettes. \[\boxed{\text{Retinoblastoma}}\]
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