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Start free with EleplanNon-functioning neuroendocrine tumor of pancreas
ORPHA:506075Disease
Also called Non-functioning PNET · Non-functioning pancreatic NET · Non-functioning pancreatic neuroendocrine tumor · Non-functioning well-differentiated NEN of pancreas · Non-functioning well-differentiated neuroendocrine neoplasm of pancreas · Non-functioning well-differentiated pancreatic NEN · Non-functioning well-differentiated pancreatic neuroendocrine neoplasm
What it is
A rare neuroendocrine tumor of pancreas characterized by a well-differentiated epithelial pancreatic neuroendocrine neoplasm measuring at least 0.5 cm, without distinct hormonal syndrome. Tumors <0.5 cm are called microadenomas. Microadenomatosis is the multifocal occurrence of microadenomas. Histopathologic examination shows an organoid growth pattern and expression of synaptophysin and chromogranin A on immunohistochemistry. Tumors are often discovered incidentally, or patients may present with symptoms related to local or metastatic tumor spread. Microadenomas are considered benign, while larger tumors may behave in a malignant manner with extrapancreatic spread, metastasis, or recurrence.
Key facts
- Age of onset
- Adult, Elderly
- Inheritance
- Not applicable
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- 1-9 / 100 000 (Japan)Neuroendocrine tumor of pancreas
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
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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