rare bile duct cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (excludes the gallbladder and ampulla of vater).

• causes obstruction early, picked up early and better prognosis

Demographics: 60-70yo, males, Asia

Risk Factors

• Primary sclerosing cholangitis (PSC).
• Chronic biliary tract inflammation.
• Chronic liver disease (i.e. chronic viral hepatitis – HBV, HCV, cirrhosis).
• Choledocholithiasis.
• Obesity.
• Liver fluke infection.
• Congenital biliary tree abnormalities (i.e. choledochal cysts – Caroli disease, congenital hepatic fibrosis).
• Genetic disorders (i.e. Lynch syndrome, multiple biliary papillomatosis).
• Environmental toxin exposure (i.e. asbestos and thorotrast).
• Intrahepatic cholangiocarcinomas are associated with mutations in the gene encoding isocitrate dehydrogenase 1 (IDH1)

Pathophysiology

Intrahepatic Cholangiocarcinoma: Located in the intrahepatic bile ducts.