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Neuroendocrine tumor of pancreas

Disease definition

Pancreatic endocrine tumor, also known as pancreatic neuroendocrine tumor (PNET), describes a group of endocrine tumors originating in the pancreas that are usually indolent and benign, but may have the potential to be malignant. They can be functional, exhibiting a hormonal hypersecretion syndrome, but can be non-functional presenting with non-specific symptoms and include insulinoma, glucagonoma, VIPoma, somatostatinoma (SSoma), PPoma and Zollinger-Ellison syndrome (ZES, or gastrinoma) and other ectopic hormone producing tumors (such as GRFoma) (see these terms).

ORPHA:97253

Classification level: Group of disorders
  • Synonym(s):
    • PNET
    • Pancreatic NET
    • Pancreatic neuroendocrine tumor
    • Well-differentiated NEN of pancreas
    • Well-differentiated neuroendocrine neoplasm of pancreas
    • Well-differentiated pancreatic NEN
    • Well-differentiated pancreatic neuroendocrine neoplasm
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable or Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: E16.8
  • OMIM: -
  • UMLS: C0242363
  • MeSH: -
  • GARD: -
  • MedDRA: -

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