{"entity":{"id":"men1-hereditary-net","kind":"term","name":"MEN1 and hereditary neuroendocrine syndromes","aka":[],"tldr":"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.","asOf":"2026-09-07","wikipedia":"https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia_type_1","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia_type_1"}],"tags":[],"related":[],"cancers":["neuroendocrine","pancreatic-net"],"sections":[],"technologies":["germline-testing"],"targets":["menin","hif2a"],"drugs":["belzutifan"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"category":"Genomics & genetics"},"route":"/terms/men1-hereditary-net/","neighbours":{"cancer":[{"id":"multiple-endocrine-neoplasia","kind":"cancer","name":"Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4)","route":"/cancers/multiple-endocrine-neoplasia/"},{"id":"men1-syndrome","kind":"cancer","name":"Multiple endocrine neoplasia type 1 (MEN1)","route":"/cancers/men1-syndrome/"},{"id":"neuroendocrine","kind":"cancer","name":"Neuroendocrine tumours","route":"/cancers/neuroendocrine/"},{"id":"pancreatic-net","kind":"cancer","name":"Pancreatic neuroendocrine tumours","route":"/cancers/pancreatic-net/"}],"technology":[{"id":"germline-testing","kind":"technology","name":"Germline (hereditary) testing","route":"/technologies/germline-testing/"}],"target":[{"id":"hif2a","kind":"target","name":"HIF-2α","route":"/targets/hif2a/"},{"id":"menin","kind":"target","name":"Menin","route":"/targets/menin/"}],"drug":[{"id":"belzutifan","kind":"drug","name":"Belzutifan","route":"/drugs/belzutifan/"}],"bottleneck":[{"id":"b-hereditary-risk","kind":"bottleneck","name":"Inherited risk is mostly unidentified","route":"/bottlenecks/b-hereditary-risk/"}],"term":[{"id":"hereditary-cancer-syndromes","kind":"term","name":"Hereditary cancer syndromes","route":"/terms/hereditary-cancer-syndromes/"}]}}