Retina: second tumours after 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: second tumours after retinoblastoma

Sample item

A man in his twenties who was treated in childhood for bilateral retinoblastoma, including external beam radiotherapy, develops a painful swelling of the femur. Which second malignancy is most strongly linked to his germline condition?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

C. Osteosarcoma

Germline RB1 mutations predispose survivors to second primary cancers, with osteosarcoma the commonest, followed by soft tissue sarcomas and melanoma. Radiotherapy raises the risk within the irradiated field, which is why it is avoided where possible. Survivors need lifelong follow-up.

Option by option

  • A. Wilms tumour

    Wilms tumour is associated with WT1 changes, not with RB1.

  • B. Hepatoblastoma

    Hepatoblastoma is an infantile tumour linked to other syndromes and not to RB1.

  • C. Osteosarcoma (correct)

    Correct. Osteosarcoma is the commonest second cancer in survivors of heritable retinoblastoma.

  • D. Acute lymphoblastic leukaemia

    Leukaemia is not the characteristic second tumour of germline RB1 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 C, Osteosarcoma. Germline RB1 mutations predispose survivors to second primary cancers, with osteosarcoma the commonest, followed by soft tissue sarcomas and melanoma.

Why is "Wilms tumour" not the answer?

Wilms tumour is associated with WT1 changes, not with RB1.

Why is "Hepatoblastoma" not the answer?

Hepatoblastoma is an infantile tumour linked to other syndromes and not to RB1.

Why is "Acute lymphoblastic leukaemia" not the answer?

Leukaemia is not the characteristic second tumour of germline RB1 disease.

What concept does this question test?

It tests Retina: second tumours after retinoblastoma in Ophthalmology. Radiotherapy raises the risk within the irradiated field, which is why it is avoided where possible. Survivors need lifelong follow-up.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. 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: second tumours after 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.