Associated conditions

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