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MEN1 — SCE Medical Oncology MCQ

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HardCancer GeneticsMEN1SCE Medical Oncology

A 45-year-old man with MEN1 syndrome (germline MEN1 mutation) undergoes regular screening. His serum calcium is elevated at 2.85 mmol/L with an inappropriately raised PTH. What is the most common endocrine tumour in MEN1 and how is it managed?

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Correct answer: C — Primary hyperparathyroidism — subtotal parathyroidectomy (3.5 glands) or total parathyroidectomy with autotransplantation

Primary hyperparathyroidism is the most common manifestation of MEN1 syndrome (occurring in >90% of patients by age 50), followed by pancreatic NETs (gastrinoma, insulinoma) and pituitary adenomas. Unlike sporadic PHPT (usually single adenoma), MEN1-associated PHPT involves multigland hyperplasia. Surgical management is subtotal (3.5 gland) parathyroidectomy or total parathyroidectomy with forearm autotransplantation. Surveillance for other MEN1 manifestations is lifelong.

Reference: ESMO 2024 NET Guidelines; MEN1 Clinical Practice Guidelines; Thakker et al JCEM 2012