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Multiple endocrine neoplasia type 1

Disease definition

Multiple endocrine neoplasia Type 1 (MEN1) is a frequent form of MEN (see this term), a rare inherited cancer syndrome, characterized by the development of neuroendocrine tumors of the parathyroid, pancreas, and anterior pituitary gland, and less commonly the adrenal cortical gland, with other non-endocrine tumors in some patients.

ORPHA:652

  • Synonym(s):
    • MEN1
    • Wermer syndrome
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant or Not applicable 
  • Age of onset: All ages
  • ICD-10: D44.8
  • OMIM: 131100
  • UMLS: C0025267
  • MeSH: D018761
  • GARD: 3829
  • MedDRA: 10028190

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