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Multiple endocrine neoplasia type 1

Key sources
The following summarized guidelines for the evaluation and management of multiple endocrine neoplasia type 1 are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2021; 2020), the American Association of Endocrine Surgeons (AAES 2016), the European Neuroendocrine Tumor Society (ENETS 2015), and the Endocrine Society (ES 2012).
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Guidelines

1.Diagnostic investigations

Genetic testing: as per ESMO 2020 guidelines, obtain genetic testing in patients with multiple endocrine neoplasias (hyperparathyroidism and/or pituitary tumours), a family history of NETs or associated diseases and features suspicious of a hereditary disease, as well as in young patients (< 40 years of age) with gastrinoma.
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2.Medical management

Setting of care
Manage patients with MEN1 and their families by a multidisciplinary team of relevant specialists with experience managing endocrine tumors.
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Ensure a multidisciplinary team approach including specialist physicians (such as endocrinologists, gastroenterologists, and oncologists), endocrine surgeons, histopathologists (with expertise in NETs), radiologists (including those with expertise in nuclear medicine), and clinical geneticists in the management of patients with NETs.
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More topics in this section

  • Management of parathyroid tumors

  • Management of pituitary tumors

  • Management of pancreatic tumors

  • Management of gastric tumors

  • Management of thymic/bronchopulmonary tumors

  • Management of adrenal tumors

3.Follow-up and surveillance

Surveillance for tumors, general principles: obtain regular screening (such as annually) for the development of MEN1-associated tumors in patients with positive MEN1 germline mutation.
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More topics in this section

  • Surveillance for tumors, parathyroid

  • Surveillance for tumors, pituitary

  • Surveillance for tumors, pancreatic

  • Surveillance for tumors, gastric

  • Surveillance for tumors, thymic/bronchopulmonary

  • Surveillance for tumors, adrenal