Bile duct cancer (cholangiocarcinoma)
Bile duct cancer (cholangiocarcinoma) is rare, with around 1,000 new cases each year in the UK. It is almost always a type of cancer called adenocarcinoma, which starts in the lining of the bile duct.
If cancer starts in the part of the bile ducts within the liver, it is known as intra-hepatic. If it starts in bile ducts outside the liver, it is known as extra-hepatic.
We hope this information answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having your treatment.
The bile ducts are tubes that carry bile. The main function of bile is to break down fats in food to help our digestion. Bile is made by the liver and stored in the gall bladder.
The bile ducts connect the liver and gall bladder to the small bowel (see diagram below). When people have had their gall bladder removed, bile flows directly from the liver into the small intestine.
The bile ducts and gall bladder are known as the biliary system.
Causes and possible risk factors of bile duct cancer
Back to top
The cause of most bile duct cancers is unknown. There are a number of risk factors that can increase your risk of developing bile duct cancer. These include:
People who have a chronic inflammatory bowel condition, known as ulcerative colitis, have an increased risk of developing this type of cancer. People who have primary sclerosing cholangitis, which is an inflammatory condition that affects the bile ducts, are also at an increased risk of developing bile duct cancer.
Abnormal bile ducts
People who are born with (congenital) abnormalities of the bile ducts, such as choledochal cysts, have a higher risk of developing bile duct cancer.
In Africa and Asia, a large number of bile duct cancers are thought to be caused by infection with a parasite known as the liver fluke.
Although bile duct cancers can occur in younger people, more than two out of three occur in people over 65.
Bile duct cancer, like other cancers, is not infectious and can't be passed on to other people.
Signs and symptoms of bile duct cancer
Back to top
Cancer in the bile ducts can block the flow of bile from the liver to the intestine. This causes bile to flow back into the blood and body tissues, and the skin and whites of the eyes to become yellow (jaundice). It also causes the urine to become a dark yellow colour and stools (bowel motions) to look pale. The skin may become itchy.
Other possible symptoms include discomfort in the tummy area (abdomen), loss of appetite, high temperatures (fevers) and weight loss.
These symptoms can be caused by many things other than bile duct cancer, but it's important to get them checked by your doctor.
How bile duct cancer is diagnosed
Back to top
Usually you begin by seeing your GP, who will examine you. They will refer you to a hospital specialist for any necessary tests, expert advice and treatment.
At the hospital, the doctor will ask you about your general health and any previous medical problems. They will also examine you and take blood samples to check your general health and that your liver is working properly.
The following tests are commonly used to diagnose bile duct cancer:
This uses sound waves to make up a picture of the bile ducts and surrounding organs. You'll usually be asked not to eat or drink anything for at least six hours before the scan. Once you’re lying comfortably on your back, a gel is spread on to your abdomen. A small device that produces sound waves is then rubbed over the area. The sound waves produce a picture on a computer. The test is painless and only takes a few minutes.
CT (computerised tomography) scan
A CT scan takes a series of x-rays that build up a three-dimensional picture of the inside of the body. The scan is painless and takes 10-30 minutes. CT scans use small amounts of radiation, which is very unlikely to hurt you or anyone you come in contact with. You will be asked not to eat or drink for at least four hours before the scan.
You may be given a drink or injection of a dye that allows particular areas to be seen more clearly. This may make you feel hot all over for a few minutes. If you’re allergic to iodine or have asthma, you could have a more serious reaction to the injection, so it's important to let your doctor know beforehand.
Spiral CT scan
In this test, the x-ray machine rotates continuously around the body to make cross-sectional pictures.
MRI (magnetic resonance imaging) scan
This test is similar to a CT scan but uses magnetism, instead of x-rays, to build up a detailed picture of areas of your body. Before the scan you may be asked to complete and sign a checklist. This is to make sure it’s safe for you to have an MRI scan.
Before having the scan, you’ll be asked to remove any metal belongings, including jewellery. Some people are given an injection of dye into a vein in the arm. This is called a contrast medium and can help images from the scan show up more clearly. During the test you will be asked to lie very still on a couch inside a long cylinder (tube) for about 30 minutes. It's painless but can be slightly uncomfortable, and some people feel a bit claustrophobic during the scan. It’s also noisy, but you’ll be given earplugs or headphones. You'll be able to hear, and speak to, the person operating the scanner.
ERCP (endoscopic retrograde cholangio-pancreatography)
This procedure may be used to take an x-ray picture of the pancreatic and bile ducts. It may also be used to unblock the bile duct, if necessary.
Your stomach and the first part of your small bowel (duodenum) need to be empty for this test. So you'll be asked not to eat or drink anything for about six hours beforehand.
You’re given an injection to relax you (a sedative) and a local anaesthetic spray to numb your throat. The doctor passes a thin, flexible tube called an endoscope into your mouth, down to your stomach and into the duodenum.
The doctor looks down the endoscope to find the opening where the bile duct and pancreatic duct drain into the duodenum. They may then inject a dye, which shows up on x-ray, into these ducts. This helps to show if there is any abnormality or blockage in the ducts.
If there is a blockage, the doctor may insert a small tube known as a stent (see below). You will be given antibiotics beforehand to help prevent any infection and you will probably stay in hospital overnight.
Endoscopic ultrasound scan (EUS)
This scan is similar to an ERCP, but involves an ultrasound probe being passed down the endoscope to take an ultrasound scan of the bile ducts and surrounding structures.
PTC (percutaneous transhepatic cholangiography)
This procedure may be used to take an x-ray picture of the bile duct. It may also be used to get a sample of tissue (biopsy) from the tumour. You will be asked not to eat or drink anything for about six hours before the test and will be given a sedative just like with an ERCP.
The doctor will numb an area on the right side of your tummy (abdomen) with a local anaesthetic injection. They will then pass a thin needle through the skin into your liver and inject a dye into the bile duct within the liver. You will have x-rays to see if there is any abnormality or blockage of the duct.
You may feel some discomfort as the needle enters the liver. You will be given antibiotics before and after the procedure to help prevent infection, and you will stay in hospital for at least one night afterwards.
This is a test to look at blood vessels. The bile duct is very close to large blood vessels, which carry blood to and from the liver. An angiogram may be used to check whether any of them are affected by the cancer.
Angiograms are carried out in the x-ray department. A fine tube is put into a blood vessel (artery) in your groin. A dye is then injected up the tube. The dye circulates in the arteries so that they show up on x-ray.
The results of the previous tests may make your doctor strongly suspect that you have cancer of the bile duct, but the only way to be sure is by having a biopsy. Some cells or tissue samples are taken from the affected area of the bile duct. The biopsy sample is then looked at under a microscope. A biopsy may be carried out during an ERCP or PTC.
CT or ultrasound may be used at the same time to make sure the biopsy is taken from the right place.
An operation called a laparotomy is sometimes used to help diagnose bile duct cancer. The operation is carried out under a general anaesthetic so you aren't awake.
The surgeon makes a cut (incision) in your abdomen to examine the bile duct and the tissue around it for cancer. A tube with a tiny camera attached called a laparoscope helps the surgeon see inside the abdomen.
If a cancer is found, but looks as though it has not spread to surrounding tissues, the surgeon may be able to remove the cancer or relieve any blockage that it’s causing.
Staging and grading of bile duct cancer
Back to top
The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing the stage of the cancer helps the doctors decide on the most appropriate treatment.
Cancer can spread in the body in the bloodstream or the lymphatic system. The lymphatic system is part of the body’s defence against infection and disease. It is made up of a network of lymph nodes connected by fine tubes. Your doctors will usually examine the lymph nodes close to the biliary system to find out the stage of your cancer.
The cancer is contained within the bile duct.
The cancer has spread through the wall of the bile duct but hasn't spread into nearby lymph nodes or other structures.
The cancer has spread into the liver, pancreas, gall bladder or nearby blood vessels, but not the lymph nodes.
The cancer has spread into nearby lymph nodes.
The cancer is affecting the main blood vessels that take blood to and from the liver, or it has spread into the small or large bowel, the stomach or the abdominal wall. Lymph nodes in the abdomen may also be affected.
The cancer has spread to distant parts of the body such as the lungs.
If the cancer comes back after treatment, it is known as recurrent cancer.
Grading refers to the appearance of cancer cells under a microscope and gives an idea of how quickly a cancer may develop.
Low-grade means that the cancer cells look very much like normal cells. They are usually slow-growing and are less likely to spread. In high-grade tumours, the cells look very abnormal, are likely to grow more quickly and are more likely to spread.
Treatment for bile duct cancer
Back to top
The treatment you have will depend on the position and size of the cancer, whether it has spread beyond the bile duct and your general health. In some situations, the aim of treatment will be to relieve symptoms.
The main treatment for bile duct cancer is surgery to remove the cancer. But this may involve a major operation and isn't always possible. The decision about whether surgery is possible and the type of operation that may be done depends on the size of the cancer and whether it has begun to spread into nearby tissues.
If surgery is recommended, you will be referred to a surgeon who is a specialist in this rare cancer.
Removal of the bile ducts
If the cancer is at a very early stage (stage 1), only the bile ducts containing the cancer are removed. The remaining ducts in the liver are then joined to the small bowel, allowing the bile to flow again.
Partial liver resection
If the cancer has begun to spread into the liver, the affected part of the liver and the bile ducts are removed.
If the cancer is larger and has spread into nearby structures, then the bile ducts, part of the stomach, part of the small bowel (duodenum), the pancreas, gall bladder and the surrounding lymph nodes are all removed.
This is a major operation. After your operation, you may stay in an intensive care ward for the first couple of days. You will then be moved to a general ward until you recover. Most people need to be in hospital for about two weeks after this type of operation.
Surgery to relieve blockage (obstruction)
If it isn't possible to remove the tumour, you may have an operation to relieve the blockage. This will also help to relieve jaundice.
The surgeon operates to create a bypass of the blocked part of the bile duct, so the bile can flow from the liver into the intestine. Another method of relieving a blockage, without an operation, is to insert a tube (stent) into the duct holding it open.
If a part of the small bowel called the duodenum is blocked, it can cause sickness (vomiting). This may be helped with an operation where the surgeon connects the stomach to the next section of small bowel (the jejunum), bypassing the duodenum.
A stent is a tube put into the bile duct to hold it open and allow bile to drain away. The tube is about as thick as a ballpoint pen refill and about 5-10cm (2-4in) long. A stent may be put in using an ERCP or occasionally a PTC procedure.
The ERCP method
The preparation and procedure is the same as for ERCP. By looking at x-rays the doctor will be able to see the narrowing in the bile duct. The narrowing is stretched using dilators (small inflatable balloons), and the stent is inserted through the endoscope so that the bile can drain. If you have any discomfort while this is being done it's important to let your doctor know.
The PTC method
The procedure and preparation is the same as for PTC. A temporary wire is passed to the blocked area and the stent is guided along the wire. Sometimes a drainage tube (catheter) is left in the bile duct. One end of the catheter is in the bile duct and the other stays outside the body connected to a bag, which collects the bile. This is to help with the insertion of the stent or, sometimes, to enable x-rays to be taken to check the position of the stent after it has been put in place. It is usually left in for a few days. Once the catheter is removed the hole heals over within two days.
Sometimes a combination of ERCP and PTC may be used.
You are given antibiotics before and after the procedure to help prevent any infection. It is likely that you will stay in hospital for a few days.
A stent usually needs to be replaced every 3-4 months to prevent it from becoming blocked. If the stent becomes blocked you may have high temperatures and/or jaundice. It's important to tell your specialist as soon as possible if you develop these symptoms. You may need antibiotic treatment and your specialist may advise that the stent be replaced. This is usually done quite easily.
Radiotherapy treats cancer by using high-energy rays to destroy cancer cells, while doing as little harm as possible to normal cells. It may be given externally from a radiotherapy machine, or internally by placing radioactive material close to the tumour (brachytherapy).
Radiotherapy may occasionally be used after surgery for bile duct cancer. This is called adjuvant radiotherapy and is given to try to reduce the risk of cancer coming back.
Radiotherapy is also sometimes used if an operation isn't possible. It can't cure the cancer but it may help shrink it or slow its growth.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. They work by disrupting the growth of cancer cells.
Chemotherapy may occasionally be used after surgery if all the cancer couldn't be removed by the operation. It may also be used if an operation isn't possible or the cancer has come back (recurred) after initial treatment. The aim of chemotherapy treatment is to try to shrink or slow down the growth of the cancer and to relieve symptoms. A combination of the drugs cisplatin and gemcitabine (Gemzar®) can be used to treat bile duct cancer.
Research is being carried out to find out if giving chemotherapy after surgery (adjuvant chemotherapy) can help to reduce the risk of cancer coming back.
Sometimes chemotherapy and radiotherapy are given together, this is called chemoradiation. It may be used after an operation to remove bile duct cancer or if the cancer can't be removed by an operation.
Photodynamic therapy (PDT)
PDT uses a combination of laser light and a light-sensitive drug to destroy cancer cells. In bile duct cancer, it is used to help relieve symptoms.
The light-sensitive drug is injected into a vein. It circulates in the bloodstream and enters cells throughout the body. The drug enters more cancer cells than healthy cells. It doesn't do anything until it is exposed to laser light of a particular wavelength.
You will have an endoscopy about two days after the injection (see ERCP). The doctor uses the endoscopy tube to shine the laser light on to the cancer. This makes the drug active and destroys the cancer cells.
You may be sensitive to light for a few days after the injection. You’ll be told what precautions you need to take until this effect wears off. Other possible side effects include pain, and inflammation or bleeding from the bile duct.
Cancer doctors use clinical trials to assess new treatments. Research is being done into possible new treatments for bile duct cancer. These include:
giving a chemotherapy drug called capecitabine after surgery to remove the cancer
using a biological treatment in combination with chemotherapy to treat advanced bile duct cancer
giving PDT at the same time as putting in a stent to relieve a blockage.
You may be invited to take part in a clinical trial. Your doctor will discuss the treatment with you so that you have a full understanding of the trial and what it means to take part.
Having investigations and treatment for cancer can be a very stressful experience.
You may have many emotions including anxiety, anger and fear. These are all normal reactions and are part of the process many people go through as they try to come to terms with their condition.
Many people find it helpful to talk things over with their doctor or nurse, or with one of our cancer support specialists. Family and close friends can also offer support.
This information has been compiled using a number of reliable sources, including:
DeVita, et al. Cancer – Principles and Practice of Oncology. 8th edition. 2008. Lippincott Williams and Wilkins.
Kelsen, et al. Gastrointestinal Oncology: Principles and Practice. 2nd edition. 2007. Lippincott Williams and Wilkins.
Khan S, et al. Cholangiocarcinoma. The Lancet. 2005. 366: 1310–14.
Photodynamic therapy for bile duct cancer. Interventional procedure guidance 134. 2005. National Institute for Health and Clinical Excellence (NICE).
Valle, et al. Cisplatin plus Gemcitabine versus Gemcitabine for Biliary Tract Cancer. New England Journal of Medicine. 2010. 362: 1273–1281.
With thanks to: Dr Richard Hubner, Consultant Medical Oncologist; Dr Andrew Webb, Consultant Medical Oncologist; and the people affected by cancer who reviewed this edition.
Reviewing is just one of the ways you could help when you join our Cancer Voices network.