# MEN1 and hereditary neuroendocrine syndromes

Source: https://onco.cc/terms/men1-hereditary-net/  
OnCo record `men1-hereditary-net` (Term). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Inherited conditions (MEN1, VHL, NF1, tuberous sclerosis) that cause neuroendocrine tumours, often multiple and at a young age, so families need genetic testing and surveillance.

## Summary

MEN1 (menin loss) causes parathyroid, pituitary and pancreatic NETs; MEN1 is also the most commonly mutated gene in sporadic pancreatic NETs (~40%), with DAXX/ATRX and mTOR-pathway genes. Germline testing is recommended for pancreatic NETs, paragangliomas (SDHx) and young-onset disease. Belzutifan is approved for VHL-associated pancreatic NETs.

## Fields

- Kind: Term
- Last checked: 2026-09-07

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia_type_1
- Wikipedia: https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia_type_1

## Connected records

- cancers: [Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4)](https://onco.cc/cancers/multiple-endocrine-neoplasia/), [Multiple endocrine neoplasia type 1 (MEN1)](https://onco.cc/cancers/men1-syndrome/), [Neuroendocrine tumours](https://onco.cc/cancers/neuroendocrine/), [Pancreatic neuroendocrine tumours](https://onco.cc/cancers/pancreatic-net/)
- technologies: [Germline (hereditary) testing](https://onco.cc/technologies/germline-testing/)
- targets: [HIF-2α](https://onco.cc/targets/hif2a/), [Menin](https://onco.cc/targets/menin/)
- drugs: [Belzutifan](https://onco.cc/drugs/belzutifan/)
- bottlenecks: [Inherited risk is mostly unidentified](https://onco.cc/bottlenecks/b-hereditary-risk/)
- terms: [Hereditary cancer syndromes](https://onco.cc/terms/hereditary-cancer-syndromes/)

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