Endocrinology and diabetes: MEN1 gene: practice question

A General Medicine 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 Medicine · Endocrinology and diabetes: MEN1 gene

Sample item

A 30-year-old man has recurrent peptic ulcers with diarrhoea, hypercalcaemia with raised PTH, and a pituitary adenoma. Mutation in which gene is most likely?

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

The answer and the reasoning

B. The gene encoding menin on chromosome 11

The combination of parathyroid hyperplasia, pancreatic neuroendocrine tumour such as gastrinoma, and a pituitary adenoma defines MEN type 1. It results from inactivating variants of the tumour suppressor MEN1, which encodes menin.

Option by option

  • A. NF1 gene encoding neurofibromin on chromosome 17

    NF1 causes neurofibromas and café-au-lait macules.

  • B. The gene encoding menin on chromosome 11 (correct)

    Correct. Parathyroid, pancreatic and pituitary tumours indicate a menin gene mutation.

  • C. VHL tumour suppressor gene on chromosome 3

    VHL causes haemangioblastoma, renal cell carcinoma and phaeochromocytoma.

  • D. RET proto-oncogene on chromosome 10

    RET mutations cause MEN type 2 with medullary thyroid carcinoma and phaeochromocytoma.

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 General Medicine question

What is the answer to this General Medicine question?

The best answer is B, The gene encoding menin on chromosome 11. The combination of parathyroid hyperplasia, pancreatic neuroendocrine tumour such as gastrinoma, and a pituitary adenoma defines MEN type 1.

Why is "NF1 gene encoding neurofibromin on chromosome 17" not the answer?

NF1 causes neurofibromas and café-au-lait macules.

Why is "VHL tumour suppressor gene on chromosome 3" not the answer?

VHL causes haemangioblastoma, renal cell carcinoma and phaeochromocytoma.

Why is "RET proto-oncogene on chromosome 10" not the answer?

RET mutations cause MEN type 2 with medullary thyroid carcinoma and phaeochromocytoma.

What concept does this question test?

It tests Endocrinology and diabetes: MEN1 gene in General Medicine. It results from inactivating variants of the tumour suppressor MEN1, which encodes menin.

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 Endocrinology and diabetes: MEN1 gene?

For each disease know the classic presentation, the best test, and the first-line treatment. Then use the flashcards and the other General Medicine 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.