Jejunal neuroendocrine tumor
Synonyms: Jejunal neuroendocrine neoplasm
- Type
- Category
- Group
- Group of disorders
- Age of onset
- Adult
- Status
- active
Jejunal neuroendocrine tumor is a rare, primary, malignant, epithelial neoplasm of the small intestine arising from enterochromaffin cells in the jejunum. Clinical behavior depends on the histologic grade, but initially it is generally characterized by vague abdominal symptoms (cramping, bloating, diarrhea) with insidious onset, although sometimes it could present with signs of bowel obstruction/perforation or gastrointestinal bleeding. Diagnosis in advanced stages with regional or distant spread is common, but signs of carcinoid syndrome (flushing, sweating, diarrhea) are usually not apparent until hepatic metastasis has occurred.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Jejunal neuroendocrine tumor | Jejunal neuroendocrine neoplasm |
| fr | Tumeur neuroendocrine du jéjunum | Néoplasme neuroendocrine du jéjunum |
| de | Tumor, neuroendokriner, des Jejunums | Jejunale neuroendokrine Neoplasie |
| es | Tumor neuroendocrino yeyunal | Neoplasia neuroendocrina yeyunal |
| it | Tumore neuroendocrino del digiuno | |
| nl | Neuro-endocriene tumor van jejunum | Jejunale neuro-endocriene tumor · Neuro-endocrien neoplasma van jejunum · Neuro-endocriene tumor van nuchtere darm |
| pt | Tumor endócrino do jejuno | |
| pl | Guz neuroendokrynny jelita czczego | Nowotwór neuroendokrynny jelita czczego |
| cs | Jejunální endokrinní nádor | = en |
| uk | Нейроендокринна пухлина підшлункової залози | Нейроендокринна пухлина підшлункової залози |
| zh | 空肠神经内分泌肿瘤 | 空肠神经内分泌瘤 |
| tr | Jejunal nöroendokrin tümör | Jejunal nöroendokrin neoplazm |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 15064 | 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:423975Neuroendocrine tumor of the small intestine
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases; rare transplant-related disorders
Children (1):
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/100077
- This record as JSON: /data/d/100077.json