Serotonin-producing neuroendocrine tumor of pancreas
Synonyms: Serotonin-producing PNET · Serotonin-producing pancreatic NET · Serotonin-producing pancreatic neuroendocrine tumor
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare functioning neuroendocrine tumor of pancreas characterized by a typically well-differentiated neoplasm composed of cells expressing serotonin. Patients may present with atypical carcinoid syndrome with abdominal pain, diarrhea, weight loss, and/or flushing. Carcinoid syndrome is usually present only when there are liver metastases. The tumors tend to be larger than non-functioning tumors and are associated with a poorer prognosis because they are almost always metastatic.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Serotonin-producing neuroendocrine tumor of pancreas | Serotonin-producing PNET · Serotonin-producing pancreatic NET · Serotonin-producing pancreatic neuroendocrine tumor |
| fr | Tumeur neuroendocrine du pancréas sécrétrice de sérotonine | PNET sécrétrice de sérotonine · Tumeur neuroendocrine pancréatique sécrétrice de sérotonine |
| de | Serotonin-produzierender neuroendokriner Tumor des Pankreas | Serotonin-produzierender NET des Pankreas · Serotonin-produzierender PNET |
| es | Tumor neuroendocrino de páncreas productor de serotonina | TNE pancreático productor de serotonina · TNEP productor de serotonina · Tumor neuroendocrino pancreático productor de serotonina |
| it | Tumore neuroendocrino pancreatico secernente serotonina | |
| nl | Serotonine-producerende neuro-endocriene tumor van pancreas | Serotonine-producerende PNET · Serotonine-producerende neuro-endocriene tumor van alvleesklier · Serotonine-producerende pancreatische NET · Serotonine-producerende pancreatische neuro-endocriene tumor |
| pt | Tumor neuroendócrino do pâncreas secretor de seretonina | |
| pl | Guz trzustki neuroendokrynny produkujący serotoninę | = en |
| cs | Neuroendokrinní tumor pankreatu produkující serotonin | PNET produkující serotonin · Pankreatický NET produkující serotonin · Pankreatický neuroendokrinní tumor produkující serotonin |
| uk | Серотонінпродукуюча нейроендокринна пухлина підшлункової залози | Серотонінпродукуюча підшлункова залоза NET · Нейроендокринна пухлина підшлункової залози, що продукує серотонін · PNET, що виробляє серотонін |
| zh | 5-羟色胺分泌型胰腺神经内分泌肿瘤 | = en |
| tr | Pankreas serotonin üreten nöroendokrin tümörü | Serotonin üreten PNET · Serotonin üreten pankreatik PNET · Serotonin üreten pankreatik nöroendokrin tümör |
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 | attributed | validated |
| MONDO | 24504 | E | validated | |
| UMLS | C0345933 | 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:506060Functioning neuroendocrine tumor 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/506090
- This record as JSON: /data/d/506090.json