Laryngeal neuroendocrine tumor
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare head and neck tumor characterized by an epithelial neoplasm with evidence of neuroendocrine differentiation, typically located in the supraglottic larynx. The tumor can be well, moderately, or poorly differentiated, the latter group being subdivided into small cell or large cell neuroendocrine carcinomas. There is a strong association with tobacco use. Patients present with hoarseness, dysphagia, sore throat, airway obstruction, hemoptysis, and rarely a paraneoplastic syndrome due to aberrant hormone production. Poorly differentiated tumors are highly aggressive with high rates of regional and distant metastasis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Laryngeal neuroendocrine tumor | |
| fr | Tumeur neuroendocrine du larynx | |
| de | Tumor, neuroendokriner, des Larynx | |
| es | Tumor neuroendocrino de laringe | |
| it | Tumore endocrino della laringe | |
| nl | Neuro-endocriene tumor van larynx | Laryngeale neuro-endocriene tumor · Neuro-endocriene tumor van strottenhoofd |
| pt | Tumor endócrino da laringe | |
| pl | Guz neuroendokrynny krtani | |
| cs | Laryngeální endokrinní nádor | |
| uk | Нейроендокринна пухлина гортані | |
| zh | 喉神经内分泌肿瘤 | |
| tr | Laringeal nöroendokrin tümör |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C32.1 | NTBT | attributed | validated |
| ICD-11 | 2F00.2 | E | specific | validated |
| MONDO | 0015070 | E | validated | |
| UMLS | C5681809 | 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:98061Rare otorhinolaryngologic tumor
rare otorhinolaryngological 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
14 annotations · validated 2017-11-13| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0100605 | Neoplasm of the larynx | |
| HP:0100634 | Neuroendocrine neoplasm | |
| Frequent (79-30%) | ||
| HP:0002039 | Anorexia | |
| HP:0012432 | Chronic fatigue | |
| HP:0002730 | Chronic noninfectious lymphadenopathy | |
| HP:0001618 | Dysphonia | |
| HP:0003528 | Elevated calcitonin | |
| HP:0002875 | Exertional dyspnea | |
| HP:0003144 | Increased serum serotonin | |
| HP:0200136 | Oral-pharyngeal dysphagia | |
| HP:0001824 | Weight loss | |
| Very rare (<4-1%) | ||
| HP:0011749 | Adrenocorticotropic hormone excess | |
| HP:0031029 | Elevated carcinoembryonic antigen level | |
| HP:0031218 | Inappropriate antidiuretic hormone secretion | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 24596175[PMID]_19536850[PMID]_2692106[PMID]_1659825[PMID]_1845268[PMID]_18417860[PMID]_23521413[PMID]_23397787[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/100083
- This record as JSON: /data/d/100083.json