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2693 pages added, reviewed or updated during the last month (last updated: 14/4/2021)


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In the majority of cases, the cause is unknown.

In the remainder, cholangiocarcinoma may be associated with:

  • primary sclerosing cholangitis, often with ulcerative colitis

  • congenital fibropolycystic disease:
    • Caroli's disease
    • congenital hepatic fibrosis
    • choledochal cyst
    • polycystic liver

  • biliary cirrhosis due to biliary atresia

  • chronic infection with Clonorchis sinensis - especially in the Far East

Last reviewed 01/2018

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