Gallbladder neuroendocrine tumor
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare, very aggressive neuroendocrine neoplasm characterized by the presence of nodular mass(es) arising from the neck, fundus or body of the gallbladder or by diffuse thickening of the gallbladder wall. Patients may be asymptomatic (diagnosed incidentally after surgical resection of the gallbladder) or may present epigastric pain, abdominal mass and/or non-specific symptoms, such as nausea, jaundice, flushing, cough, wheezing, ascites, and anepithymia. Paraneoplastic syndromes, such as Cushing syndrome, hypercalcemia, acanthosis nigricans, bullous pemphigoid, dermatomyositis and the Leser-Trélat sign, may be associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Gallbladder neuroendocrine tumor | |
| fr | Tumeur neuroendocrine de la vésicule biliaire | |
| de | Tumor, neuroendokriner, der Gallenblase | |
| es | Tumor neuroendocrino de la vesícula biliar | |
| it | Tumore neuroendocrino della cistifellea | |
| nl | Neuro-endocriene tumor van galblaas | |
| pt | Tumor endócrino da vesícula biliar | |
| pl | Guz neuroendokrynny woreczka żółciowego | |
| cs | Endokrinní nádor žlučníku | |
| uk | Нейроендокринна пухлина жовчного міхура | |
| zh | 胆囊神经内分泌肿瘤 | |
| tr | Safra kesesi nöroendokrin tümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C23 | NTBT | attributed | validated |
| ICD-11 | 2C13.Y | NTBT | index | validated |
| UMLS | C3273115 | E | validated | |
| GARD | 19761 | 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 (2):
- ORPHA:100101Neuroendocrine tumor with other location
rare endocrine diseases; rare neoplastic diseases - ORPHA:306633Rare tumor of gallbladder and extrahepatic biliary tract
rare hepatic 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
18 annotations · validated 2017-11-13| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0100574 | Biliary tract neoplasm | |
| HP:0100634 | Neuroendocrine neoplasm | |
| Frequent (79-30%) | ||
| HP:0003270 | Abdominal distention | |
| HP:0002039 | Anorexia | |
| HP:0001541 | Ascites | |
| HP:0005230 | Biliary tract obstruction | |
| HP:0001082 | Cholecystitis | |
| HP:0012432 | Chronic fatigue | |
| HP:0002730 | Chronic noninfectious lymphadenopathy | |
| HP:0010638 | Elevated alkaline phosphatase of hepatic origin | |
| HP:0030948 | Elevated gamma-glutamyltransferase level | |
| HP:0002574 | Episodic abdominal pain | |
| HP:0012334 | Extrahepatic cholestasis | |
| HP:0001046 | Intermittent jaundice | |
| HP:0002018 | Nausea | |
| HP:0001824 | Weight loss | |
| Very rare (<4-1%) | ||
| HP:0012658 | Abnormal brain FDG positron emission tomography | |
| HP:0004375 | Neoplasm of the nervous system | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 26576584[PMID]_20375728[PMID]_26000067[PMID]_25910356[PMID]_25525590[PMID]_25043774[PMID]_24666880[PMID]_24455579[PMID]_23826575[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/100086
- This record as JSON: /data/d/100086.json