ORPHA:424996
Squamous cell carcinoma of gallbladder and extrahepatic biliary tract
Synonyms: Squamous cell carcinoma of gallblader and EBT
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare hepatic and biliary tract tumor, arising either in the gallbladder itself or in the epithelium lining the extrahepatic biliary tree, the cystic duct and peribiliary glands. It is characterized by a substantial keratinization with abundant keratohyalin pearls and central deposition of dense keratin material within infiltrative nests and locally aggressive nature. In the early stages of the disease symptoms are vague and nonspecific (abdominal pain, jaundice and vomiting). In the advanced stages it may present with a bulky tumor and symptoms of adjacent organ involvement.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Squamous cell carcinoma of gallbladder and extrahepatic biliary tract | Squamous cell carcinoma of gallblader and EBT |
| fr | Carcinome épidermoïde de la vésicule biliaire et des voies biliaires extrahépatiques | Carcinome épidermoïde de la vésicule biliaire et des VBEH |
| de | Plattenepithelkarzinom der Gallenblase und extrahepatischen Gallengänge | Squamöses Karzinom der Gallenblase und extrahepatischen Gallengänge |
| es | Carcinoma de células escamosas de vesícula biliar y vías biliares extrahepáticas | Carcinoma de células escamosas de vesícula y VBE |
| it | Carcinoma a cellule squamose della cistifellea e delle vie biliari extraepatiche | Carcinoma a cellule squamose della cistifellea e delle VBE |
| nl | Plaveiselcelcarcinoom van galblaas en extrahepatische galwegen | Plaveiselcelcarcinoom van galblaas en EBT |
| pt | Carcinoma de células escamosas da vesícula biliar e do tracto biliar extra-hepático | Carcinoma de células escamosas da vesícula biliar e do TBEH |
| pl | Rak płaskonabłonkowy pęcherzyka żółciowego i zewnątrzwątrobowego przewodu żółciowego | Rak płaskonabłonkowy pęcherzyka żółciowego i EBT |
| cs | Dlaždicobuněčný karcinom žlučníku a extrahepatálních žlučových cest | Dlaždicobuněčný karcinom žlučníku a EBT |
| uk | Плоскоклітинний рак жовчного міхура та позапечінкових жовчовивідних шляхів | Плоскоклітинний рак жовчного міхура та EBT |
| zh | 胆囊和肝外胆道鳞状细胞癌 | 胆囊鳞状细胞癌和EBT |
| tr | Safra kesesi ve ekstrahepatik safra yollarının skuamöz hücreli karsinomu | Safra kesesi ve EBT'nin skuamöz hücreli karsinomu |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C23 | BTNT | attributed | validated |
| ICD-10 | C24.0 | BTNT | attributed | validated |
| ICD-10 | C24.1 | BTNT | attributed | validated |
| ICD-10 | C24.8 | BTNT | attributed | validated |
| ICD-10 | C24.9 | BTNT | attributed | validated |
| MONDO | 0018537 | E | validated | |
| UMLS | C4707898 | 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
23 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.032 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.026 / 100 000 | Austria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.006 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.027 / 100 000 | Bulgaria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.025 / 100 000 | Croatia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.124 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.037 / 100 000 | Estonia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.026 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.018 / 100 000 | France | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.055 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.043 / 100 000 | Iceland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.018 / 100 000 | Ireland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.037 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.005 / 100 000 | Latvia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.033 / 100 000 | Lithuania | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.016 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.015 / 100 000 | Poland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.002 / 100 000 | Portugal | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.077 / 100 000 | Slovakia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.042 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.051 / 100 000 | Switzerland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.014 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.021 / 100 000 | United Kingdom | 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/424996
- This record as JSON: /data/d/424996.json