Bronchial neuroendocrine tumor
Synonyms: Bronchial NET
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare neuroendocrine neoplasm characterized by origin from pulmonary neuroendocrine cells and ranging from low-grade typical carcinoid and intermediate-grade atypical carcinoid to high-grade large-cell neuroendocrine carcinoma and small-cell carcinoma. Two thirds of the tumors are located in the major bronchi, with a predilection for the right lung, in particular the middle lobe. Most patients with central bronchial tumors present with hemoptysis, cough, recurrent pulmonary infections, fever, chest discomfort, and unilateral wheezing, while peripheral carcinoids are usually discovered only incidentally. Carcinoid syndrome or Cushing syndrome are very rare. The tumors may be part of multiple endocrine neoplasia type 1.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Bronchial neuroendocrine tumor | Bronchial NET |
| fr | Tumeur neuroendocrine bronchique | |
| de | Tumor, neuroendokriner, bronchialer | Bronchialer NET |
| es | Tumor neuroendocrino bronquial | TNE bronquial |
| it | Tumore neuroendocrino dei bronchi | NET bronchiale |
| nl | Bronchiale neuro-endocriene tumor | Bronchiale NET |
| pt | Tumor endócrino brônquico | |
| pl | Guz endokrynny oskrzeli | = en |
| cs | Bronchiální endokrinní nádor | Karcinoid bronchů · Neuroendokrinní nádor bronchů |
| uk | Нейроендокринна пухлина бронхів | Бронхіальна мережа |
| zh | 支气管神经内分泌肿瘤 | 支气管NET |
| tr | Bronşiyal nöroendokrin tümör | Bronşiyal NET |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C34.9 | NTBT | attributed | validated |
| ICD-11 | 2C25.Y | NTBT | index | validated |
| MONDO | 0019963 | E | validated | |
| MONDO | 19963 | E | validated | |
| UMLS | C1334452 | 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:101945Rare bronchopulmonary and pleural cavity tumors
rare respiratory 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
31 annotations · validated 2017-11-08| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0030445 | Pulmonary carcinoid tumor | |
| Frequent (79-30%) | ||
| HP:0006530 | Abnormal pulmonary interstitial morphology | |
| HP:0002039 | Anorexia | |
| HP:0002099 | Asthma | |
| HP:0100749 | Chest pain | |
| HP:0002730 | Chronic noninfectious lymphadenopathy | |
| HP:0002094 | Dyspnea | |
| HP:0002105 | Hemoptysis | |
| HP:0031246 | Nonproductive cough | |
| HP:0002090 | Pneumonia | |
| HP:0004396 | Poor appetite | |
| HP:0001824 | Weight loss | |
| HP:0030828 | Wheezing | |
| Very rare (<4-1%) | ||
| HP:0031566 | Abnormal pulmonary valve cusp morphology | |
| HP:0012701 | Bowel urgency | |
| HP:4000007 | Bronchoconstriction | |
| HP:0030149 | Cardiogenic shock | |
| HP:0001005 | Dermatological manifestations of systemic disorders | |
| HP:0000845 | Elevated circulating growth hormone concentration | |
| HP:0007380 | Facial telangiectasia | |
| HP:0001399 | Hepatic failure | |
| HP:0002240 | Hepatomegaly | |
| HP:0002615 | Hypotension | |
| HP:0003154 | Increased circulating ACTH level | |
| HP:0003118 | Increased circulating cortisol level | |
| HP:0003144 | Increased serum serotonin | |
| HP:0030166 | Night sweats | |
| HP:0001962 | Palpitations | |
| 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: 27373769[PMID]_18473355[PMID]_2992742[PMID]_12371583[PMID]_22112810[PMID]_11073802[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97287
- This record as JSON: /data/d/97287.json