Neuroendocrine tumor of pancreas
Synonyms: 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
- Type
- Category
- Group
- Group of disorders
- Inheritance
- Autosomal dominant, Not applicable
- Age of onset
- Adult
- Status
- active
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).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neuroendocrine tumor of pancreas | 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 |
| fr | Tumeur neuroendocrine du pancréas | NEN du pancréas bien différencié · NEN pancréatique bien différencié · Néoplasme neuroendocrine du pancréas bien différencié · Néoplasme neuroendocrine pancréatique bien différencié · PNET |
| de | Neuroendokriner Tumor des Pankreas | Hoch-differenzierte NEN des Pankreas · Hoch-differenzierte neuroendokrine Neoplasie des Pankreas · Hoch-differenzierte pankreatische neuroendokrine Neoplasie · NET des Pankreas · Neuroendokrine Neoplasie des Pankreas · PNET |
| es | Tumor neuroendocrino del páncreas | Neoplasia neuroendocrina del páncreas bien diferenciada · Neoplasia neuroendocrina pancreática bien diferenciada · PNET · TNE bien diferenciado del páncreas · TNE bien diferenciado pancreático · TNE pancreático · Tumor neuroendocrino pancreático |
| it | Tumore neuroendocrino del pancreas | Tumore neuroendocrino pancreatico · NEN ben differenziato del pancreas · NET pancreatico · PNET · Tumore neuroendocrino ben differenziato del pancreas |
| nl | Neuro-endocriene tumor van pancreas | Goed gedifferentieerd NEN van pancreas · Goed gedifferentieerd neuro-endocrien neoplasma van pancreas · Goed gedifferentieerd pancreatisch NEN · Goed gedifferentieerd pancreatisch neuro-endocrien neoplasma · Neuro-endocriene pancreastumor · Neuro-endocriene tumor van alvleesklier · PNET · Pancreatische NET · Pancreatische neuro-endocriene tumor |
| pt | Tumor pancreático endócrino | |
| pl | Guz neuroendokrynny trzustki | = en |
| cs | Endokrinní nádory pankreatu | Neuroendokrinní nádory pankreatu |
| uk | Нейроендокринна пухлина підшлункової залози | Панкреатична NET · Нейроендокринна пухлина підшлункової залози · PNET · Добре диференційований НЕН підшлункової залози · Добре диференційована нейроендокринна пухлина підшлункової залози · Добре диференційований панкреатичний НЕН · Добре диференційована нейроендокринна пухлина підшлункової залози |
| zh | 胰腺神经内分泌肿瘤 | 高分化胰腺神经内分泌肿瘤 |
| tr | Pankreasın nöroendokrin tümörü | PNET · Pankreatik NET · Pankreatik nöroendokrin tümörü · İyi-diferansiye pankreatik NEN · Pankreasın iyi-diferansiye NEN'i · İyi-diferansiye pankreatik nöroendokrin neoplazmı · İyi-diferansiye pankreasın nöroendokrin neoplazmı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0019954 | E | validated | |
| UMLS | C4305467 | E | validated | |
| GARD | 13034 | E | validated | |
| MedDRA | 10067517 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:506052Neuroendocrine neoplasm of pancreas
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
7 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.22 / 100 000 | United States | Value and class | Validated | 18515795[PMID]_National Cancer OTHERitute[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.27 / 100 000 | Japan | Value and class | Validated | 24499825[PMID] |
| Point prevalence | 1-9 / 100 000 | 2.69 / 100 000 | Japan | Value and class | Validated | 24499825[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.21 / 100 000 | France | Value and class | Validated | 15016750[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.21 / 100 000 | Europe | Value and class | Validated | 15016750[PMID] |
| Point prevalence | 1-5 / 10 000 | 27.5 / 100 000 | United States | Value and class | Validated | 23237225[PMID]_[EXPERT] |
| Point prevalence | 1-5 / 10 000 | — | Europe | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97253
- This record as JSON: /data/d/97253.json