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Start free with EleplanNeuroendocrine tumor of pancreas
ORPHA:97253Category
Also called 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
What it is
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).
Key facts
- Prevalence
- 1-9 / 100 000 (Japan)
- Age of onset
- Adult
- Inheritance
- Autosomal dominant, Not applicable
- Classified as
- Category
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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