Breast and endocrine surgery: paraganglioma genetics: 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 · Breast and endocrine surgery: paraganglioma genetics

Sample item

A 30-year-old man has a large retroperitoneal paraganglioma with liver metastases. Mutation in which gene is most strongly associated with malignant behaviour in these tumours?

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

The answer and the reasoning

C. SDHB

Mutations of the succinate dehydrogenase subunit B gene (SDHB) are associated with a high risk of malignant, extra-adrenal paragangliomas. RET mutations cause MEN 2 and are usually associated with benign phaeochromocytoma, and VHL and NF1 are typically associated with benign adrenal tumours.

Option by option

  • A. VHL

    VHL tumours are mainly benign and noradrenaline-secreting.

  • B. RET

    RET mutation causes MEN 2 with mostly benign adrenal phaeochromocytomas.

  • C. SDHB (correct)

    Correct. SDHB carriers have the highest malignant potential.

  • D. NF1

    NF1-related phaeochromocytomas are usually benign and adrenal.

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 Surgery question

What is the answer to this General Surgery question?

The best answer is C, SDHB. Mutations of the succinate dehydrogenase subunit B gene (SDHB) are associated with a high risk of malignant, extra-adrenal paragangliomas.

Why is "VHL" not the answer?

VHL tumours are mainly benign and noradrenaline-secreting.

Why is "RET" not the answer?

RET mutation causes MEN 2 with mostly benign adrenal phaeochromocytomas.

Why is "NF1" not the answer?

NF1-related phaeochromocytomas are usually benign and adrenal.

What concept does this question test?

It tests Breast and endocrine surgery: paraganglioma genetics in General Surgery. RET mutations cause MEN 2 and are usually associated with benign phaeochromocytoma, and VHL and NF1 are typically associated with benign adrenal tumours.

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 Breast and endocrine surgery: paraganglioma genetics?

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.