Neoplasia: paediatric renal tumours: practice question

A Pathology 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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Pathology · Neoplasia: paediatric renal tumours

Sample item

A 3-year-old boy has aniridia, genitourinary anomalies and developmental delay. He is at increased risk of which tumour, and which chromosomal region is deleted?

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

The answer and the reasoning

A. Wilms tumour; 11p13

The WAGR syndrome comprises Wilms tumour, aniridia, genitourinary anomalies and intellectual disability (formerly mental retardation), caused by a deletion at 11p13 involving WT1 and PAX6. Affected children need regular renal ultrasound surveillance.

Option by option

  • A. Wilms tumour; 11p13 (correct)

    Deletion of 11p13 involving WT1 predisposes to Wilms tumour in WAGR syndrome.

  • B. Neuroblastoma; 2p24 amplification

    Neuroblastoma is linked to MYCN amplification and is not part of WAGR syndrome.

  • C. Rhabdomyosarcoma; t(2;13)

    Alveolar rhabdomyosarcoma carries the PAX3-FOXO1 fusion and is not part of the syndrome.

  • D. Retinoblastoma; 13q14

    Retinoblastoma is linked to deletions of the RB1 gene at 13q14 and is unrelated to aniridia.

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 Pathology question

What is the answer to this Pathology question?

The best answer is A, Wilms tumour; 11p13. The WAGR syndrome comprises Wilms tumour, aniridia, genitourinary anomalies and intellectual disability (formerly mental retardation), caused by a deletion at 11p13 involving WT1 and PAX6.

Why is "Neuroblastoma; 2p24 amplification" not the answer?

Neuroblastoma is linked to MYCN amplification and is not part of WAGR syndrome.

Why is "Rhabdomyosarcoma; t(2;13)" not the answer?

Alveolar rhabdomyosarcoma carries the PAX3-FOXO1 fusion and is not part of the syndrome.

Why is "Retinoblastoma; 13q14" not the answer?

Retinoblastoma is linked to deletions of the RB1 gene at 13q14 and is unrelated to aniridia.

What concept does this question test?

It tests Neoplasia: paediatric renal tumours in Pathology. Affected children need regular renal ultrasound surveillance.

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 Neoplasia: paediatric renal tumours?

Learn each disease as a bundle: cause, key histology, markers, and the clinical hallmark. Then use the flashcards and the other Pathology 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.