Neuroendocrine neoplasm of esophagus
Synonyms: Esophageal NEN · Esophageal neuroendocrine neoplasm · NEN of esophagus
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A group of esophageal epithelial neoplasms characterized by neuroendocrine differentiation, comprising well-differentiated neuroendocrine tumors (NETs), poorly differentiated neuroendocrine carcinomas (NECs), and mixed neuroendocrine-non-neuroendocrine neoplasms, an umbrella category including mixed adenoneuroendocrine carcinoma. The tumors typically occur in the lower esophagus, often in association with Barrett mucosa. NECs may also arise in other parts of the esophagus. On endoscopy, NETs usually appear as small polypoid or nodular submucosal masses, while NECs are large, infiltrative, and ulcerated. Patients most commonly present with dysphagia, pain, weight loss, and sometimes melena. Metastatic NETs may be associated with carcinoid syndrome.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neuroendocrine neoplasm of esophagus | Esophageal NEN · Esophageal neuroendocrine neoplasm · NEN of esophagus |
| fr | Néoplasme neuroendocrine de l'oesophage | NEN de l'oesophage |
| de | Neuroendokrine Neoplasie des Ösophagus | NEN des Oesophagus · Neoplasie, neuroendokrine oesophageale |
| es | Neoplasia neuroendocrina del esófago | NEN del esófago · NEN esofágica · Neoplasia neuroendocrina esofágica |
| it | Neoplasia neuroendocrina dell'esofago | |
| nl | Neuro-endocrien neoplasma van oesofagus | NEN van oesofagus · Neuro-endocrien neoplasma van slokdarm · Oesofageaal NEN · Oesofageaal neuro-endocrien neoplasma |
| pt | Neoplasia neuroendócrina do esófago | |
| pl | Rak neuroendokrynny przełyku | = en |
| cs | Neuroendokrinní novotvar jícnu | NEN jícnu |
| uk | Нейроендокринне новоутворення стравоходу | Стравохідний NEN · Нейроендокринна пухлина стравоходу · NEN стравоходу |
| zh | 食管神经内分泌肿瘤 | 小肠神经内分泌肿瘤 |
| tr | Özofagus nöroendokrin neoplazmı | Özofageal NEN · Özofagusa ait NEN · Özofagus nöroendokrin neoplazmı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C15.8 | NTBT | attributed | validated |
| ICD-11 | 2B70.Y | NTBT | index | validated |
| MONDO | 0003649 | E | validated | |
| UMLS | C1333462 | 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:180821Rare gastroesophageal tumor
rare gastroenterological 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/506136
- This record as JSON: /data/d/506136.json