Genetic and metabolic disorders: Peutz-Jeghers syndrome: practice question

A Pediatrics 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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Pediatrics · Genetic and metabolic disorders: Peutz-Jeghers syndrome

Sample item

A 9-year-old has dark brown macules on the lips and buccal mucosa and recurrent colicky abdominal pain. At laparotomy for obstruction, an intussusception is found with a polyp as the lead point. What is the histological nature of these polyps?

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

The answer and the reasoning

C. Hamartomatous with a branching smooth muscle core

Peutz-Jeghers syndrome is an autosomal dominant STK11 disorder with mucocutaneous pigmentation and hamartomatous gastrointestinal polyps, mostly in the small bowel. Polyps cause bleeding and intussusception, and patients carry an increased lifetime cancer risk.

Option by option

  • A. Inflammatory polyps from chronic colitis

    Inflammatory polyps occur in inflammatory bowel disease and do not accompany lip pigmentation.

  • B. Adenomatous with dysplastic epithelium

    Adenomatous polyps characterise familial adenomatous polyposis and do not accompany mucosal pigmentation.

  • C. Hamartomatous with a branching smooth muscle core (correct)

    Correct. The polyps are hamartomas with an arborising smooth muscle core, and they cause intussusception.

  • D. Lymphoid hyperplasia from viral infection

    Lymphoid hyperplasia can lead intussusception after a viral illness but does not accompany pigmented macules.

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 Pediatrics question

What is the answer to this Pediatrics question?

The best answer is C, Hamartomatous with a branching smooth muscle core. Peutz-Jeghers syndrome is an autosomal dominant STK11 disorder with mucocutaneous pigmentation and hamartomatous gastrointestinal polyps, mostly in the small bowel.

Why is "Inflammatory polyps from chronic colitis" not the answer?

Inflammatory polyps occur in inflammatory bowel disease and do not accompany lip pigmentation.

Why is "Adenomatous with dysplastic epithelium" not the answer?

Adenomatous polyps characterise familial adenomatous polyposis and do not accompany mucosal pigmentation.

Why is "Lymphoid hyperplasia from viral infection" not the answer?

Lymphoid hyperplasia can lead intussusception after a viral illness but does not accompany pigmented macules.

What concept does this question test?

It tests Genetic and metabolic disorders: Peutz-Jeghers syndrome in Pediatrics. Polyps cause bleeding and intussusception, and patients carry an increased lifetime cancer risk.

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 Genetic and metabolic disorders: Peutz-Jeghers syndrome?

Memorise milestones and the vaccine schedule as tables and revise them often. Then use the flashcards and the other Pediatrics 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.