AD 6/02
Cholangiocarcinoma (Klatskin tumor)
Epidemiology: 2000-3000 cases per year. Accounts for 3% of GI malignancies. Usually presents between
ages 50-70 but can present earlier in pts with primary sclerosing cholangitis (PSC) and in pts with
choledochal cysts. Slightly higher incidence in men.
Pathogenesis : From the intrahepatic and extrahepatic epithelial cells. Some thought that related to
mutation in tumor suppressor gene.
Risk factors:
• Primary sclerosing cholangitis: Recall that incidence of ulcerative colitis is 90% in pts with PSC. 30%
of cholangiocarcinomas are diagnosed in pts with PSC and UC, and lifetime risk in these patients is
10-15%. Unclear why these patients are at greater risk but smoking and alcohol may contribute.
• Choledochal cysts: Risk is related to duration of disease. May be as high as 15% per year after first
two decades.
• Parasitic infections: Liver flukes (Clonorchis and Opisthorchis) are associated with intrahepatic
cholangiocarcinoma; may be due to inflammatory response.
• Hepatolithiasis (also called Oriental cholangiohepatitis) is rare in US but present in up to 50% in
Taiwanese and other Asians with gallstones.
• Toxin exposures: auto, rubber, chemical, and wood-finishing occupations
• Genetics: “cancer family" syndromes (eg, Lynch, Li-Fraumeni), Caroli’s syndrome (congenital dilation
of intrahepatic ducts), multiple biliary papillomatosis
Clinical presentation:
• Symptoms: pruritis 60%, abdl pain 30-50%, weight loss 30-50%, fever 20%, fatigue, clay stools ,
dark urine. Classic triad for hepatobiliary or pancreatic cancer is cholestasis, abdl pain, weight loss.
• Signs: jaundice (often intermittent) 90%, hepatomegaly 25-40%
• Lab test: increased bilirubin, increased alkaline phosphatase.
--CEA elevated in some but not sensitive or specific. Best if level obtained directly from bile.
--CA 19-9 elevated in 80% and most helpful in pts with PSC. Combining the two tests is more helpful.
Radiographic imaging