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Peutz-Jeghers Syndrome — SCE Medical Oncology MCQ

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HardCancer GeneticsPeutz-Jeghers SyndromeSCE Medical Oncology

A well 34-year-old STK11 pathogenic-variant carrier with Peutz-Jeghers syndrome asks for annual pancreatic MRI and EUS despite no pancreatic-cancer family history. What is the current UK position?

Educational content. Not a substitute for clinical judgement or local policy.

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Correct answer: D — Offer pancreatic surveillance only within a formal ethically approved research study; continue established PJS surveillance

Explanation lettering: E = shown as C · C = shown as E

D is correct. PJS increases pancreatic-cancer risk, but NHS Genomics Education records the UKCGG/EHTG position that evidence of survival benefit is insufficient for routine pancreatic surveillance outside an ethically approved study. That does not negate established PJS surveillance: luminal gastrointestinal and small-bowel assessment and very-high-risk breast screening remain important. CA19-9 is not a screening gatekeeper, prophylactic pancreatectomy is disproportionate, and pancreatic imaging does not replace prevention of polyp-related intussusception or bleeding. The answer distinguishes elevated risk from proof that a screening intervention improves outcomes.

Reference: NHS Genomics Education Peutz-Jeghers guidance: https://www.genomicseducation.hee.nhs.uk/genotes/knowledge-hub/peutz-jeghers-syndrome/