Orphanet: Glucagonoma

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Disease definition

Glucagonoma is a rare, functioning type of pancreatic neuroendocrine tumor (PNET; see this term) that hypersecretes glucagon, leading to a syndrome comprised of necrolytic migratory erythema, diabetes mellitus, anemia, weight loss, mucosal abnormalities, thromboembolism, gastrointestinal and neuropsychiatric symptoms.


Classification level: Disorder
  • Synonym(s):
    • Glucagonoma syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: E16.8
  • OMIM: -
  • UMLS: C0017689
  • MeSH: D005935
  • GARD: 2496  9399
  • MedDRA: 10018404

Detailed information


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