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Tumours of the Biliary Tract

Tumours of the Biliary Tract

Tumours of the biliary tract (ie, the gallbladder and bile ducts) are relatively uncommon, accounting for about two per cent of all cancers. The occurrence of cancers of the bile duct and gallbladder in the UK is about 2.8 cases per 100,000 females and about 2.0 cases per 100,000 men (ie, it is rarer in men).

Cancers of the biliary tract include:

  • Cholangiocarcinomas: cancers arising from the linings of bile ducts including intrahepatic bile duct cancer (that starts in the ducts in the liver), common bile duct cancer (that starts in the common bile duct), Klatskin tumours (or perihilar cancers, that start where the left and right hepatic ducts join), and extrahepatic cancer (that starts in the bile ducts outside of the liver)
  • Cancer of the gallbladder (a very rare condition – affecting only about 500 people in the UK each year, mainly people over the age of seventy)
  • Cancer of the ampulla of Vater (another rare malignant tumour that starts near the end of the common bile duct and causes an obstruction)

Cholangiocarcinomas are the most usual form of primary (ie, original site) tumour in the bile ducts.

Benign (non-cancerous) tumours, such as adenomas, also occur in the biliary tract but are rare (more frequently found in women).

Tumours in the biliary tract are usually small which means they are particularly hard to see with standard imaging techniques.

Although all these cancers are rare, a family history of congenital fibrosis or cysts means you are at greater risk of contracting them. Having gallstones is also a risk factor (nearly everyone with gallbladder cancer has gallstones), so too is exposure to certain toxins and drugs, including oral contraceptives. More than two out of three cases of bile cancer occur in people over the age of 65. Obesity may also increase the risk of bile duct cancer.

Symptoms

When tumours do arise in the biliary tract they are serious, particularly as they rarely produce symptoms or signs in the early stages of development. Later, jaundice is likely to be apparent when the tumour blocks the bile ducts (which can also be blocked by gallstones). Such blockages typically become more regular as the condition develops. As symptoms worsen, infection is likely.

Other symptoms include pruritis (itching), loss of appetite and weight-loss, and difficulties stopping blood loss through skin cuts (known as coagulopathy), fevers, and pains in the right hand side of the ribs (ie, where the liver is).

Diagnosis

Liver function tests are likely to indicate if there is a problem. This test will be supported by diagnostic imaging such as ultrasound, MRI or CT scans. The bile ducts themselves can also be X-rayed using a procedure known as a cholangiogram (or ERCP) which can be used to unblock the bile duct if necessary.

Laparotomy (a small incision in the tummy performed under general anaesthetic) is sometimes used to help diagnose bile duct cancer. During the procedure, which is performed under a general anaesthetic, the surgeon may also remove the cancer (if it has not spread to another site) or relieve the blockage.

Treatment

Treatment options vary depending on the exact site, size and location of the tumour. Surgery is the only effective treatment. This is a major operation and unfortunately not all patients will be able to have an operation to remove the tumour. Where a surgical procedure is performed it may be followed by (adjuvant) radiotherapy or chemotherapy treatment in some cases.

Different surgical procedures may be performed depending on the size and the cancer and where it has spread.

Procedures include:

  • Removal of the bile ducts
  • Cholecystectomy – usually a laparoscopic operation to remove the whole gallbladder. This may be appropriate surgery for gallbladder cancer if the cancer is limited to the gallbladder itself. An alternative to the laparoscopic procedure is an open procedure that removes the whole of the gallbladder, some of the liver tissue and all surrounding lymph nodes. This extended procedure is designed to try to remove cancer in these surrounding tissues as well
  • Radical gallbladder section if the cancer has spread outside of the gallbladder
  • Liver resection (if the cancer has spread from the ducts to the liver)
  • Whipple’s procedure (a very major operation)

Radio and chemotherapy treatments may also be given if surgery is not possible to help slow down the spread of cancer (or shrink the size of the tumour). Sometimes these treatments may be combined (chemoradiation).

Prognosis/outlook

Patients with intrahepatic cholangiocarcinomas have poor prognosis as the tumour spreads early to other parts of the body. Benign (and malignant) tumours can reappear even after they have been removed by surgery. Occasionally initially benign tumours can develop into malignant growths.

Prognosis is much better for patients with extrahepatic tumours who are suitable for early surgical intervention.

Treatment of the symptoms is usually possible, for example surgery to remove the blockage rather than the tumour (which relieves the jaundice). This kind of treatment can improve and extend the quality of life of some patients.