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Ophthalmology · Retina: trilateral retinoblastoma
Sample itemA 3-year-old child treated for bilateral retinoblastoma now has persistent morning vomiting, headache and drowsiness. MRI shows a midline mass near the third ventricle. Which tumour is this?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Pineoblastoma
Heritable retinoblastoma can occur with a primitive neuroectodermal tumour in the pineal region, a combination called trilateral retinoblastoma. It presents with raised intracranial pressure from hydrocephalus and carries a poor outlook. Children with germline disease are therefore followed with neuroimaging.
Option by option
A. Medulloblastoma of the vermis
Medulloblastoma arises in the posterior fossa and is not linked to the RB1 gene in this way.
B. Craniopharyngioma
Craniopharyngioma is a suprasellar cystic tumour that causes chiasmal compression and endocrine problems.
C. Optic pathway glioma
Optic pathway glioma is associated with neurofibromatosis type 1, not with retinoblastoma.
D. Pineoblastoma (correct)
Correct. A pineal primitive neuroectodermal tumour with bilateral retinoblastoma defines trilateral disease.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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