Highlights & Basics
- Multiple endocrine neoplasia syndromes are hereditary tumor syndromes with distinct patterns of organ involvement.
- Mutations in the MEN1 gene typically cause type 1 multiple endocrine neoplasia (MEN1), and mutations in the RET proto-oncogene typically cause type 2 multiple endocrine neoplasia (MEN2). MEN2 is divided into subtypes, MEN2A and MEN2B (also known as MEN2 and MEN3). MEN4 is a very rare disease, caused by mutations in the CDKN1B gene.
- Prophylactic thyroidectomy in childhood is indicated in MEN2.
- Medical management of hormonal hypersecretion is important for symptom control.
- Most tumors require surgical evaluation, although surgical cure is not always possible.
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History & Exam
Key Factors
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Definition
Epidemiology
Etiology
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Citations
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Wells SA Jr, Asa SL, Dralle H, et al. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. Thyroid. 2015 Jun;25(6):567-610.[Abstract][Full Text]
Al-Salameh A, Cadiot G, Calender A, et al. Clinical aspects of multiple endocrine neoplasia type 1. Nat Rev Endocrinol. 2021 Apr;17(4):207-24.[Abstract][Full Text]
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