Retina: trilateral retinoblastoma: practice question

A Ophthalmology question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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Ophthalmology · Retina: trilateral retinoblastoma

Sample item

A 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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Questions about this Ophthalmology question

What is the answer to this Ophthalmology question?

The best answer is D, Pineoblastoma. Heritable retinoblastoma can occur with a primitive neuroectodermal tumour in the pineal region, a combination called trilateral retinoblastoma.

Why is "Medulloblastoma of the vermis" not the answer?

Medulloblastoma arises in the posterior fossa and is not linked to the RB1 gene in this way.

Why is "Craniopharyngioma" not the answer?

Craniopharyngioma is a suprasellar cystic tumour that causes chiasmal compression and endocrine problems.

Why is "Optic pathway glioma" not the answer?

Optic pathway glioma is associated with neurofibromatosis type 1, not with retinoblastoma.

What concept does this question test?

It tests Retina: trilateral retinoblastoma in Ophthalmology. It presents with raised intracranial pressure from hydrocephalus and carries a poor outlook. Children with germline disease are therefore followed with neuroimaging.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Retina: trilateral retinoblastoma?

Group the red eye by what to examine and what the pupil does. Then use the flashcards and the other Ophthalmology questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.