Classic neuroendocrine tumor of appendix
Synonyms: Classic appendiceal neuroendocrine tumor · Classic appendix neuroendocrine tumor
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare endocrine tumor of the appendix, seen twice as frequently in females than in males, and usually presenting before the fifth decade of life. It is usually asymptomatic when located in the tip of the appendix (without obstruction), but acute appendicitis is often associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Classic neuroendocrine tumor of appendix | Classic appendiceal neuroendocrine tumor · Classic appendix neuroendocrine tumor |
| fr | Tumeur neuroendocrine classique de l'appendice | Tumeur neuroendocrine appendiculaire classique |
| de | Neuroendokriner Tumor des Apendix, klassischer | Appendix-NET, klassischer |
| es | Tumor neuroendocrino clásico del apéndice | Tumor neuroendocrino apendicular clásico |
| it | Tumore endocrino classico dell'appendice | Tumore endocrino appendicolare classico |
| nl | Klassieke neuro-endocriene tumor van appendix | Klassieke appendiculaire neuro-endocriene tumor |
| pt | Tumor endócrino clássico do apêndice | Tumor neuroendócrino clássico do apêndice |
| pl | Klasyczny guz neuroendokrynny wyrostka robaczkowego | = en |
| cs | Klasický endokrinní nádor apendixu | = en |
| uk | Класична нейроендокринна пухлина апендикса | Класична нейроендокринна пухлина апендикса · Класична нейроендокринна пухлина апендикса |
| zh | 经典阑尾神经内分泌肿瘤 | = en |
| tr | Apendiksin klasik nöroendokrin tümörü | Klasik apendiks nöroendokrin tümör · Klasik apendiks nöroendokrin tümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D37.3 | NTBT | attributed | validated |
| MONDO | 0018016 | E | validated | |
| UMLS | C5679854 | 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:100079Neuroendocrine neoplasm of appendix
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.25 / 100 000 | Worldwide | Value and class | Not yet validated | 20524865[PMID] |
| Point prevalence | Unknown | — | Worldwide | 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/329977
- This record as JSON: /data/d/329977.json