Cholangiocarcinoma

Cholangiocarcinoma is an adenocarcinoma that may arise from anywhere in the biliary tree from small intra-hepatic bile ducts to the common bile duct.

Most occur at the porta hepatis. Distal tumours obstruct main ducts and present early; intrahepatic tumours present late and may be mistaken for hepatocellular carcinoma.

In the UK, there are approximately 20 new cases per 1,000,000 per year.

Cholangiocarcinoma is more common in males because of the predominant occurrence of primary sclerosing cholangitis (PSC) in men

The prognosis is poor:

• 5-year survival of less than 5%
• median survival for patients with intrahepatic cholangiocarcinoma is expected to be 18-30 months. A slightly shorter survival has been reported for patients with perihilar cholangiocarcinoma (12-24 months)
• cure can only be expected from surgical resection of early stage tumour
  • after surgery, the 5-year recurrence rate is in the range of 60-90%. Since most patients initially present with advanced disease, they are not amenable to primary surgical resection; 75% of patients die within 1 year of diagnosis
• death is usually due to hepato-cellular failure and infection.

Reference:

• 1. Khan SA et al. Cholangiocarcinoma. Lancet 2005;366:1303-1314.