Paediatric urology: Wilms tumour: practice question

A General Surgery 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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General Surgery · Paediatric urology: Wilms tumour

Sample item

A 3-year-old child has a large smooth flank mass that does not cross the midline, hypertension and haematuria. He has aniridia. Which gene is implicated in this association?

ARB1 on chromosome 13q14BAPC on chromosome 5q21CMYCN amplificationDWT1 on chromosome 11p13

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

The answer and the reasoning

D. WT1 on chromosome 11p13

Wilms tumour (nephroblastoma) is the commonest paediatric renal malignancy. WAGR syndrome results from a deletion at 11p13 involving WT1 and PAX6, giving Wilms tumour, aniridia, genitourinary anomalies and retardation. Treatment is chemotherapy with nephrectomy, and the prognosis is good.

Option by option

A. RB1 on chromosome 13q14
RB1 mutation causes retinoblastoma and osteosarcoma.
B. APC on chromosome 5q21
APC mutation causes familial adenomatous polyposis.
C. MYCN amplification
MYCN amplification is a poor prognostic marker of neuroblastoma, whose mass crosses the midline.
D. WT1 on chromosome 11p13 (correct)
WT1 deletion at 11p13 is linked to Wilms tumour with aniridia (WAGR syndrome).

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.

Questions about this General Surgery question

What is the answer to this General Surgery question?

The best answer is D, WT1 on chromosome 11p13. Wilms tumour (nephroblastoma) is the commonest paediatric renal malignancy.

Why is "RB1 on chromosome 13q14" not the answer?

RB1 mutation causes retinoblastoma and osteosarcoma.

Why is "APC on chromosome 5q21" not the answer?

APC mutation causes familial adenomatous polyposis.

Why is "MYCN amplification" not the answer?

MYCN amplification is a poor prognostic marker of neuroblastoma, whose mass crosses the midline.

What concept does this question test?

It tests Paediatric urology: Wilms tumour in General Surgery. WAGR syndrome results from a deletion at 11p13 involving WT1 and PAX6, giving Wilms tumour, aniridia, genitourinary anomalies and retardation. Treatment is chemotherapy with nephrectomy, and the prognosis is good.

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 Paediatric urology: Wilms tumour?

Learn classic signs and eponyms alongside what they diagnose. Then use the flashcards and the other General Surgery 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.