ORPHA:424975
Squamous cell carcinoma of liver and intrahepatic biliary tract
Synonyms: Squamous cell carcinoma of liver and IBT
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Squamous cell carcinoma of liver and intrahepatic biliary tract is an extremely rare, primary, malignant liver and biliray tract epithelial tumor originating in the intrahepatic bile duct epithelium histologically characterized by the presence of keratinization and/or intracellular bridges. Patients typically present abdominal pain in the right upper quadrant, jaundice, nausea, vomiting, anorexia, weight loss, fever and/or dyspepsia.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Squamous cell carcinoma of liver and intrahepatic biliary tract | Squamous cell carcinoma of liver and IBT |
| fr | Carcinome épidermoïde du foie et des voies biliaires intrahépatiques | Carcinome épidermoïde du foie et des VBIH |
| de | Plattenepithelkarzinom der Leber und intrahepatischen Gallengänge | Squamöses Karzinom der Leber und intrahepatischen Gallengänge |
| es | Carcinoma de células escamosas de hígado y vías biliares intrahepáticas | Carcinoma carcinoide de hígado y VBI |
| it | Carcinoma a cellule squamose del fegato e delle vie biliari intraepatiche | Carcinoma a cellule squamose del fegato e delle VBI |
| nl | Plaveiselcelcarcinoom van lever en intrahepatische galwegen | Plaveiselcelcarcinoom van lever en IBT |
| pt | Carcinoma de células escamosas hepático e do tracto biliar intra-hepático | Carcinoma de células escamosas do fígado e do TBIH |
| pl | Rak płaskonabłonkowy wątroby i wewnątrzwątrobowego przewodu żółciowego | Rak płaskonabłonkowy wątroby i IBT |
| cs | Dlaždicobuněčný karcinom jater a intrahepatálních žlučových cest | Dlaždicobuněčný karcinom jater a IBT |
| uk | Плоскоклітинний рак печінки та внутрішньопечінкових жовчних шляхів | Плоскоклітинний рак печінки та IBT |
| zh | 肝和肝内胆道鳞状细胞癌 | 肝鳞状细胞癌和IBT |
| tr | Karaciğer ve intrahepatik safra yollarının skuamöz hücreli karsinomu | Karaciğer ve IBT'nin skuamöz hücreli karsinomu |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C22.0 | BTNT | attributed | validated |
| ICD-10 | C22.1 | BTNT | attributed | validated |
| MONDO | 0018534 | E | validated | |
| UMLS | C4749400 | 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:424936Carcinoma of liver and intrahepatic 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
18 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.009 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.016 / 100 000 | Latvia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.004 / 100 000 | Lithuania | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.003 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.005 / 100 000 | Poland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.006 / 100 000 | Portugal | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.003 / 100 000 | Slovakia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.017 / 100 000 | Switzerland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.002 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.014 / 100 000 | United Kingdom | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.009 / 100 000 | Austria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.003 / 100 000 | Bulgaria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.018 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.009 / 100 000 | Estonia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.007 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.008 / 100 000 | France | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.004 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.019 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
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
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/424975
- This record as JSON: /data/d/424975.json