Neuroendocrine tumor of anal canal
Synonyms: NET of anal canal
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult, Elderly
- Status
- active
A are epithelial tumor of the anal canal arising from enterochromaffin cells in the colorectal-type epithelium above the dentate line and in the anal transition zone. The tumors are slow growing and the majority of cases are diagnosed in later advanced stages. It may present with symptoms related to the anatomical location of the tumor (rectal mass, rectal bleeding and pain, tenesmus or changes in bowel habits), symptoms of carcinoid syndrome (flushing and increased gut motility) or nonspecific symptoms of advanced disease (hepatomegaly, fever, weight loss, anorexia, malaise).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neuroendocrine tumor of anal canal | NET of anal canal |
| fr | Tumeur neuroendocrine du canal anal | NET du canal anal |
| de | Tumor, neuroendokriner, des Analkanals | NET des Analkanals |
| es | Tumor neuroendocrino del canal anal | TNE del canal anal |
| it | Tumore neuroendocrino del canale anale | |
| nl | Neuro-endocriene tumor van anaal kanaal | NET van anaal kanaal |
| pt | Tumor endócrino anal | |
| pl | Guz neuroendokrynny kanału odbytu | NET kanału odbytu · Nowotwór neuroendokrynny kanału odbytu |
| cs | Endokrinní nádor análního kanálu | = en |
| uk | Нейроендокринна пухлина анального каналу | НЕП анального каналу |
| zh | 肛管神经内分泌肿瘤 | 肛管NET |
| tr | Anal kanalın nöroendokrin tümörü | NET anal kanal |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C21.1 | NTBT | attributed | validated |
| ICD-11 | 2C00.2 | E | specific | validated |
| MONDO | 0015069 | E | validated | |
| UMLS | C3272849 | 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:424010Epithelial tumor of anal canal
rare gastroenterological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:481508Gastroenteric neuroendocrine neoplasm
rare endocrine 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
25 annotations · validated 2017-11-08| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0100570 | Carcinoid tumor | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0002039 | Anorexia | |
| HP:0030446 | Atypical pulmonary carcinoid tumor | |
| HP:0025085 | Bloody diarrhea | |
| HP:0002730 | Chronic noninfectious lymphadenopathy | |
| HP:0002019 | Constipation | |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0002573 | Hematochezia | |
| HP:0002240 | Hepatomegaly | |
| HP:0030144 | Hypoactive bowel sounds | |
| HP:0012702 | Tenesmus | |
| HP:0001824 | Weight loss | |
| Very rare (<4-1%) | ||
| HP:0031566 | Abnormal pulmonary valve cusp morphology | |
| HP:0012701 | Bowel urgency | |
| HP:4000007 | Bronchoconstriction | |
| HP:0007380 | Facial telangiectasia | |
| HP:0002615 | Hypotension | |
| HP:0003144 | Increased serum serotonin | |
| HP:0030145 | Lack of bowel sounds | |
| HP:0002249 | Melena | |
| 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: 18490556[PMID]_20664474[PMID]_24163193[PMID]_24763982[PMID]_24412088[PMID]_16913162[PMID]_22052063[PMID]_1568162[PMID]_15213627[PMID]_24468036[PMID]_27175651[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/100082
- This record as JSON: /data/d/100082.json