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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