Phys · endocrine
Multiple Endocrine Neoplasia
Also known as Multiple Endocrine Neoplasia · multiple endocrine neoplasia · MEN1 · MEN2 · MEN2A · MEN2B · Wermer syndrome · Sipple syndrome · medullary thyroid carcinoma · pheochromocytoma · gastrinoma · insulinoma · Zollinger-Ellison syndrome
Consultant-physician depth guide to multiple endocrine neoplasia — MEN1 (MENIN mutation, primary hyperparathyroidism, pancreatic neuroendocrine tumours including gastrinoma and insulinoma, pituitary adenoma) and MEN2 (RET proto-oncogene, medullary thyroid cancer, pheochromocytoma, primary hyperparathyroidism), ATA genotype-risk stratification, prophylactic thyroidectomy timing, calcitonin screening, biochemical surveillance, and cascade genetic testing. Structured for FRACP DWE and DCE preparation.
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Multiple Endocrine Neoplasia
Meet the patient
Meet a 34-year-old man with known MEN1 (a germline MEN1 mutation), status post subtotal parathyroidectomy at 22, now on omeprazole for a gastrinoma, presenting in the emergency department with renal colic and a serum calcium of 2.95 mmol per litre. The question is never just "treat the calcium" — it is which of his endocrine glands is failing now, and in what order to act on them.[1]
Four questions frame every MEN case: what is the syndrome pattern and its genetic basis, what is the correct sequence of operations, how does the genotype time prophylactic surgery, and what is the lifelong surveillance programme? Hold those four and the long case answers itself.[1][2]
You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Physician Medicine fellowship atlas.
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- [1]Brandi ML, Pieterman CRC, English KA, et al. Multiple endocrine neoplasia type 1 (MEN1): recommendations and guidelines for best practice. Lancet Diabetes Endocrinol, 2025.PMID 40523372
- [2]Thakker RV, Newey PJ, Walls GV, et al. Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab, 2012.PMID 22723327
- [3]English KA, Pieterman CRC, Marini F, et al. Treatments for MEN1-associated endocrine tumours: three systematic reviews and a meta-analysis. Lancet Diabetes Endocrinol, 2025.PMID 40523371
- [4]Schreinemakers JM, Pieterman CR, Scholten A, et al. The optimal surgical treatment for primary hyperparathyroidism in MEN1 patients: a systematic review. World J Surg, 2011.PMID 21713580
- [5]Ito T, Igarashi H, Uehara H, et al. Causes of death and prognostic factors in multiple endocrine neoplasia type 1: a prospective study: comparison of 106 MEN1/Zollinger-Ellison syndrome patients with 1613 literature MEN1 patients with or without pancreatic endocrine tumors. Medicine (Baltimore), 2013.PMID 23645327
- [6]Brandi ML, Agarwal SK, Perrier ND, et al. Multiple Endocrine Neoplasia Type 1: Latest Insights. Endocr Rev, 2021.PMID 33249439
- [7]Amodru V, Taieb D, Guerin C, et al. MEN2-related pheochromocytoma: current state of knowledge, specific characteristics in MEN2B, and perspectives. Endocrine, 2020.PMID 32388798
- [8]Frank-Raue K, Raue F Hereditary Medullary Thyroid Cancer: Genotype-Phenotype Correlation. Recent Results Cancer Res, 2025.PMID 40102258
- [9]Febrero B, Rodríguez JM, Ríos A, et al. Prophylactic thyroidectomy in multiple endocrine neoplasia 2 (MEN2) patients with the C634Y mutation: A long-term follow-up in a large single-center cohort. Eur J Surg Oncol, 2019.PMID 30366876
- [10]Bae YJ, Schaab M, Kratzsch J Calcitonin as Biomarker for the Medullary Thyroid Carcinoma. Recent Results Cancer Res, 2025.PMID 40102257
- [11]Rossi RE, Elvevi A, Citterio D, et al. Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies. World J Gastroenterol, 2021.PMID 34629807
- [12]West CE, Mirshahi UL, Ruth KS, et al. Medullary Thyroid Cancer Risk and Mortality in Carriers of Incidentally Identified MEN2A RET Variants. JAMA Netw Open, 2025.PMID 40577012