What is Cholangiocarcinoma?

(Cholangiocarcinoma is pronounced kol-an-gee-oh-car-sin-oh-ma).

Cholangiocarcinoma, or bile duct cancer, is a primary liver cancer that arises within the biliary system.  It can occur in the small bile ducts within the liver, the big branches in the centre of the liver (hilum) or in the main or common bile duct draining out of the liver.

Where is the liver?

The liver is found just below the right lung and is protected from injury by the lower ribs.

What does the liver do?

The liver is one of the most complex organs in the body. It’s like a “chemical factory” as it performs hundreds of tasks that are needed to keep the body healthy and alive, including:

  • Fighting infection and disease
  • Removing poisons and drugs (including alcohol)
  • Cleaning the blood
  • Controlling the amount of cholesterol
  • Storing vitamins, minerals and sugar
  • Processing food once it has been digested
  • Helping to control blood clotting (thickening)
  • Releasing bile1, the liquid that breaks down fats and helps digestion

The biliary system explained

The biliary system includes the network, or tree-like structure, of ducts within the liver and the common bile duct outside the liver.

The biliary system explainedWithin the liver the smaller ducts, similar to capillaries, drain bile1 from the cells of the liver into larger and larger branches. The right and left hepatic bile ducts inside the liver collect the bile, and join outside the liver to form the common bile duct (extrahepatic bile duct), which carries the bile from the liver to the gall bladder and down to the small intestine.

The gallbladder acts as a reservoir to hold bile until food reaches the small intestine. It is attached by a small duct, called the cystic duct, to the common bile duct about one-third of the way down the common bile duct from the liver.

1Bile is made in the liver and stored in the gallbladder. It helps to break down fats found in foods, and to rid the body of waste material filtered out of the bloodstream by the liver.


Cholangiocarcinoma may affect any part of the bile ducts

Cholangiocarcinoma may affect any part of the bile ducts

Depending on where the cancer begins, cholangiocarcinoma is categorized as follows:

Intrahepatic – affecting bile ducts within the liver.

Extrahepatic – affecting the bile duct outside the liver.

Hilar (Perihilar or Klatskin tumours) – affecting the junction of the left and right hepatic ducts.

Distal – affecting the bottom portion of the bile duct, near to the connection with the small intestine and pancreas

Adenocarcinoma, cancer arising from the mucus glands lining the inside of the bile ducts, is the most common type of cholangiocarcinoma, accounting for up to 95% of all cases.

Gall bladder Cancer – is another type of cholangiocarcinoma which develops inside the gall bladder.

Incidence of cholangiocarcinoma

The incidence of cholangiocarcinoma is increasing throughout the world, with the NCIN/Cancer52 ‘Rare and Less Common Cancers’ report2 showing the latest figure for 2013 for England only to be 1,965 reported cases, and the 2013 figure for gall bladder cancer to be 751 cases, again for England only.

2 To see an extract from the NCIN/Cancer52 Rare and Less Common Cancers report showing the ICD-10 coding, incidence and mortality data for the Upper Gastrointestinal cancers, including cholangiocarcinoma, for the individual years 2010-2013, click here


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November 2015