ORPHA:99978
Perihilar cholangiocarcinoma
Synonyms: Hilar CCA · Hilar cholangiocarcinoma · Klatskin tumor
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Klatskin tumor is an extra-hepatic cholangiocarcinoma (CCA) arising in the junction of the main right or left hepatic ducts to form the common hepatic duct.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Perihilar cholangiocarcinoma | Hilar CCA · Hilar cholangiocarcinoma · Klatskin tumor |
| fr | Cholangiocarcinome périhilaire | CCA hilaire · Cholangiocarcinome hilaire · Tumeur de Klatskin |
| de | Cholangiokarzinom, perihiläres | Hiläres Gallengangkarzinom · Klatskin-Tumor · Perihiläres CCA |
| es | Colangiocarcinoma perihiliar | CCA hiliar · Colangiocarcinoma hiliar · Tumor de Klatskin |
| it | Tumore di Klatskin | Colangiocarcinoma ilare · CCA ilare |
| nl | Perihilair cholangiocarcinoom | Hilair CCA · Hilair cholangiocarcinoom · Klatskin-tumor |
| pt | Colangiocarcinoma peri-hilar | CCA hilar · Colangiocarcinoma hilar · Tumor de Klatskin |
| pl | Guz Klatskina | Hilar CCA · Hilar cholangiocarcinoma |
| cs | Klatskinův nádor | Hilový CCA · Hilový cholangiokarcinom |
| uk | Пухлина Клацкіна | Хилярна CCA · Хилярна холангіокарцинома |
| zh | 克拉斯金肿瘤 | 肝门部胆管癌 |
| tr | Klatskin tümörü | Hilar CCA · Hiler kolanjiyokarsinom |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C24.0 | NTBT | attributed | validated |
| ICD-11 | 2C18.0 | E | specific | validated |
| MONDO | 0003345 | E | validated | |
| MeSH | D018285 | E | validated | |
| UMLS | C0206702 | E | validated | |
| GARD | 10175 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:56044Carcinoma of gallbladder and extrahepatic biliary tract
rare hepatic diseases; rare neoplastic diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 1.5 / 100 000 | United States | Value and class | Not yet validated | 19638920[PMID]_ORPHANET |
| Point prevalence | 1-9 / 100 000 | — | Europe | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
10 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0030153 | Cholangiocarcinoma | |
| HP:0012334 | Extrahepatic cholestasis | |
| HP:0000952 | Jaundice | |
| Frequent (79-30%) | ||
| HP:0002240 | Hepatomegaly | |
| HP:0002716 | Lymphadenopathy | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0004936 | Venous thrombosis | |
| HP:0001824 | Weight loss | |
Run the differential on the 3 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99978
- This record as JSON: /data/d/99978.json