Neuroendocrine carcinoma of pancreas
Synonyms: Pancreatic NEC · Pancreatic neuroendocrine carcinoma · Poorly-differentiated NEN of pancreas · Poorly-differentiated neuroendocrine neoplasm of pancreas · Poorly-differentiated pancreatic NEN · Poorly-differentiated pancreatic neuroendocrine neoplasm
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare neuroendocrine neoplasm of pancreas characterized by a high-grade malignant epithelial tumor with neuroendocrine differentiation. Based on histopathologic appearance, a small cell (composed of diffuse sheets of cells) and a large cell type (showing a nesting/trabecular pattern) are distinguished. Synaptophysin and chromogranin are positive on immunohistochemistry. The Ki-67 proliferation index is typically very high (>60 - 80%). Patients present with back pain, jaundice, and/or non-specific abdominal symptoms. Serum hormone activity is unusual. The tumor is highly aggressive with poor prognosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neuroendocrine carcinoma of pancreas | Pancreatic NEC · Pancreatic neuroendocrine carcinoma · Poorly-differentiated NEN of pancreas · Poorly-differentiated neuroendocrine neoplasm of pancreas · Poorly-differentiated pancreatic NEN · Poorly-differentiated pancreatic neuroendocrine neoplasm |
| fr | Carcinome neuroendocrine du pancréas | Carcinome neuroendocrine pancréatique · NEN du pancréas peu différencié · NEN pancréatique peu différencié · Néoplasme neuroendocrine du pancréas peu différencié · Néoplasme neuroendocrine pancréatique peu différencié |
| de | Neuroendokrines Karzinom des Pankreas | Niedrig-differenzierte NEN des Pankreas · Niedrig-differenzierte PNEN · Niedrig-differenzierte neuroendokrine Neoplasie des Pankreas · PanNEC |
| es | Carcinoma neuroendocrino de páncreas | CNE pancreático · Carcinoma neuroendocrino pancreático · NEN de páncreas pobremente diferenciada · NEN pancreática pobremente diferenciada · Neoplasia neuroendocrina de páncreas pobremente diferenciada · Neoplasia neuroendocrina pancreática pobremente diferenciada |
| it | Carcinoma neuroendocrino del pancreas | |
| nl | Neuro-endocrien carcinoom van pancreas | Slecht gedifferentieerd pancreatisch NEN · Slecht gedifferentieerd pancreatisch neuro-endocrien neoplasma · Neuro-endocrien carcinoom van alvleesklier · Pancreatisch NEC · Pancreatisch neuro-endocrien carcinoom · Slecht gedifferentieerd NEN van pancreas · Slecht gedifferentieerd neuro-endocrien neoplasma van pancreas |
| pt | Carcinoma neuroendócrino do pâncreas | |
| pl | Rak neuroendokrynny trzustki | = en |
| cs | Neuroendokrinní karcinom pankreatu | Pankreatický NEC · Pankreatický neuroendokrinní karcinom · Špatně diferencovaný pankreatický NEN · Špatně diferencovaný pankreatický neuroendokrinní novotvar · Špatně diferencovaný neuroendokrinní novotvar pankreatu |
| uk | Нейроендокринна карцинома підшлункової залози | Панкреатична NEC · Нейроендокринна карцинома підшлункової залози · Погано диференційована NEN підшлункової залози · Погано диференційована нейроендокринна пухлина підшлункової залози · Погано диференційована панкреатична NEN · Погано диференційована нейроендокринна пухлина підшлункової залози |
| zh | 胰腺神经内分泌癌 | 低分化胰腺神经内分泌肿瘤 |
| tr | Pankreas nöroendokrin karsinomu | Pankreatik NEC · Pankreatik nöroendokrin karsinom · Kötü-diferansiye pankreatik NEN · Pankreasın kötü-diferansiye NEN'i · Kötü diferansiye pankreatik nöroendokrin neoplazmı · Pankreasın kötü diferansiye nöroendokrin neoplazmı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C25.4 | NTBT | attributed | validated |
| ICD-11 | 2C10.1 | NTBT | index | validated |
| MONDO | 0005893 | E | validated | |
| MONDO | 5893 | E | validated | |
| UMLS | C2205506 | 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
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/506098
- This record as JSON: /data/d/506098.json