Neuroendocrine tumor of stomach
Synonyms: GNET · Gastric NET · Gastric neuroendocrine tumor · NET of stomach
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare subtype of neuroendocrine neoplasm, arising from enterochromaffin-like cells in the stomach, with a variable clinical presentation, disease course and prognosis, depending on the disease type and histological grade. Most patients are asymptomatic, with diagnosis usually occurring incidentally during gastroscopy, however, symptoms of dyspepsia, anemia, pain, weight loss and gastrointestinal bleeding can be observed. Association with Zollinger-Ellison syndrome and multiple endocrine neoplasia type I has been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neuroendocrine tumor of stomach | GNET · Gastric NET · Gastric neuroendocrine tumor · NET of stomach |
| fr | Tumeur neuroendocrine de l'estomac | Tumeur neuroendocrine gastrique |
| de | Tumor, neuroendokriner, des Magens | GNET · Gastraler NET · Gastraler neuroendokriner Tumor · NET des Magens |
| es | Tumor neuroendocrino de estómago | TNE de estómago · TNE gástrico · TNEG · Tumor neuroendocrino gástrico |
| it | Tumore neuroendocrino dello stomaco | |
| nl | Neuro-endocriene tumor van maag | GNET · NET van maag · Neuro-endocriene maagtumor |
| pt | Tumor endócrino gástrico | |
| pl | Guz neuroendokrynny żołądka | GNET |
| cs | Žaludeční endokrinní nádor | = en |
| uk | Нейроендокринна пухлина шлунка | Gastric NET · Нейроендокринна пухлина шлунка · GNET · НЕП шлунку |
| zh | 胃神经内分泌肿瘤 | 胃神经内分泌瘤 |
| tr | Mide nöroendokrin tümörü | GNET · Mide ağı · Mide NET · Gastrik nöroendokrin tümör |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C16.9 | NTBT | attributed | validated |
| ICD-11 | 2B72.1 | NTBT | index | validated |
| MONDO | 0003111 | E | validated | |
| UMLS | C1333783 | 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:481508Gastroenteric neuroendocrine neoplasm
rare endocrine diseases; rare neoplastic diseases - ORPHA:63443Rare epithelial tumor of stomach
rare gastroenterological diseases; rare neoplastic diseases
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:481469OBSOLETE: Gastric neuroendocrine tumor type 1 referred to · inactive
- ORPHA:481475OBSOLETE: Gastric neuroendocrine tumor type 2 referred to · inactive
- ORPHA:481478OBSOLETE: Gastric neuroendocrine tumor type 3 referred to · inactive
- ORPHA:481481OBSOLETE: Gastric neuroendocrine tumor type 4 referred to · inactive
Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| ATRX | Candidate gene tested in | Not yet assessed | HGNC:886 | ENSG00000085224 | 300032 | Xq21.1 | ATRX chromatin remodeler | 26026117[PMID] |
Prevalence and incidence
4 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 3.2 / 100 000 | Europe | Value and class | Validated | 25480460[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.7 / 100 000 | United States | Value and class | Validated | 25480460[PMID] |
| Point prevalence | 1-9 / 1 000 000 | 0.7 / 100 000 | United Kingdom | Value and class | Validated | 25480460[PMID] |
| Point prevalence | 1-9 / 1 000 000 | 0.5 / 100 000 | Japan | Value and class | Validated | 25480460[PMID] |
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
32 annotations · validated 2017-11-08| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0100570 | Carcinoid tumor | |
| Frequent (79-30%) | ||
| HP:0002039 | Anorexia | |
| HP:0002574 | Episodic abdominal pain | |
| HP:0002254 | Intermittent diarrhea | |
| HP:0001891 | Iron deficiency anemia | |
| HP:0002017 | Nausea and vomiting | |
| HP:0004396 | Poor appetite | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0030446 | Atypical pulmonary carcinoid tumor | |
| HP:0025085 | Bloody diarrhea | |
| HP:0002730 | Chronic noninfectious lymphadenopathy | |
| HP:0001005 | Dermatological manifestations of systemic disorders | |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0002248 | Hematemesis | |
| HP:0002240 | Hepatomegaly | |
| HP:0030145 | Lack of bowel sounds | |
| HP:0002249 | Melena | |
| HP:0002044 | Zollinger-Ellison syndrome | |
| Very rare (<4-1%) | ||
| HP:0031566 | Abnormal pulmonary valve cusp morphology | |
| HP:0012701 | Bowel urgency | |
| HP:4000007 | Bronchoconstriction | |
| HP:0030149 | Cardiogenic shock | |
| HP:0007380 | Facial telangiectasia | |
| HP:0001399 | Hepatic failure | |
| HP:0002615 | Hypotension | |
| HP:0003154 | Increased circulating ACTH level | |
| HP:0003144 | Increased serum serotonin | |
| HP:0001962 | Palpitations | |
| HP:0002668 | Paraganglioma | |
| HP:0004385 | Protracted diarrhea | |
| HP:0001708 | Right ventricular failure | |
| HP:0005180 | Tricuspid regurgitation | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 24415864[PMID]_22052063[PMID]_9070484[PMID]_18097129[PMID]_18097129[PMID]_22997445[PMID]_22262004[PMID]_18941127[PMID]_26511392[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/100075
- This record as JSON: /data/d/100075.json