What is the birth-to-age-50 tumor risk in MEN1 mutation carriers?

By age 50: primary hyperparathyroidism 90%+, enteropancreatic NETs 80–100% (gastrinoma 40–60%, insulinoma 10%, non-functional pNETs), pituitary adenoma 30–40% (prolactinoma most common). Annual surveillance with fasting gut hormones, calcium, and MRI is essential from age 5–8.

When is parathyroid surgery performed in MEN1?

Subtotal parathyroidectomy (3.5 glands) is recommended when calcium is clearly elevated or symptomatic. Timing is debated: operating earlier reduces hypercalcemia complications but carries higher recurrence risk. Pre-operative localization with Sestamibi/CT is less reliable than in sporadic disease due to multigland disease.

MEN1 Syndrome Tumor Probability Calculator

Estimate probability of MEN1-associated tumors (parathyroid adenoma, gastrinoma, insulinoma, pituitary adenoma) by age and mutation status to guide biochemical and imaging surveillance.

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MEN1 Syndrome Tumor Probability Calculator

Frequently Asked Questions

What is the birth-to-age-50 tumor risk in MEN1 mutation carriers?

When is parathyroid surgery performed in MEN1?