Bile duct cancer (cholangiocarcinoma)

Bile duct cancer (cholangiocarcinoma) is a type of cancer that starts in the lining of the bile duct.

The bile ducts are part of the digestive system. Bile breaks down fats in food to help us digest them.

Symptoms of bile duct cancer include:

  • the skin and whites of the eyes turning yellow (jaundice)
  • urine turning dark yellow
  • stools (bowel motions) looking pale
  • itchy skin.

You will have tests to diagnose bile duct cancer. You might have scans, including an ultrasound, CT or MRI scan. Doctors may also need to collect tissue samples (a biopsy) from the affected area of the bile duct. A biopsy can be taken in different ways.

Surgery, chemotherapy and radiotherapy are all possible treatments for bile duct cancer.

What is bile duct cancer?

Bile duct cancer (cholangiocarcinoma) is a rare cancer that starts in the lining of the bile duct.

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

The bile ducts are part of the digestive system. They are the tubes that connect the liver and gall bladder to the small bowel. Bile ducts carry bile, which breaks down fats in food to help us digest them. Bile is made by the liver and stored in the gall bladder. The bile ducts and gall bladder together are known as the biliary system.

The position of the bile ducts
The position of the bile ducts

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Bile duct cancer can affect any part of the bile ducts. These ducts are named depending on their position in the body:

  • intra-hepatic – the bile ducts inside the liver
  • extra-hepatic – the bile ducts outside the liver.

The extra-hepatic bile ducts are divided into two parts:

  • hilar – where the left and right hepatic ducts meet, just below the liver
  • distal – the lower part of the bile ducts, nearest to the bowel.


Causes and risk factors of bile duct cancer

The causes of most bile duct cancers are unknown. But there are some factors that can increase your risk of developing it.

Age

Although younger people can have bile duct cancer, most people are diagnosed over the age of 60.

Inflammatory conditions

Some long-term inflammatory conditions can increase the risk of bile duct cancer. These are:

  • ulcerative colitis – which causes inflammation of the bowel
  • primary sclerosing cholangitis – a rare condition which causes inflammation of the bile ducts.

Abnormal bile ducts

People born with bile duct abnormalities, such as choledochal cysts, have a higher risk of developing bile duct cancer.

Chronic liver disease

People who have scarring of the liver (cirrhosis) have an increased risk of developing bile duct cancer. There are many different causes of cirrhosis, such as hepatitis B and C, fatty liver disease, or alcohol-related liver disease.

Infection

In Africa and Asia, a large number of bile duct cancers are thought to be caused by infection with a parasite called the liver fluke.

Bile duct cancer, like other cancers, is not infectious. It cannot be passed on to other people.


Signs and symptoms of bile duct cancer

Cancer in the bile ducts can block the flow of bile from the liver to the bowel. This means that the bile flows back into the blood and body tissues. The main symptoms of this can include:

  • the skin and whites of the eyes turning yellow (jaundice)
  • urine turning dark yellow
  • stools (bowel motions) looking pale
  • itchy skin.

Other possible symptoms include:

  • discomfort in the tummy area (abdomen)
  • loss of appetite
  • tiredness
  • feeling generally unwell
  • high temperatures (fevers)
  • weight loss.

These symptoms can be caused by many things other than bile duct cancer. But it is important to get them checked by your doctor.


Diagnosing bile duct cancer

You usually begin by seeing your GP, who will examine you and may take a blood test. They will refer you to a hospital specialist.

At the hospital, the doctor will ask you about your symptoms 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 doctor might use the following tests to diagnose bile duct cancer.

Ultrasound scan

This uses soundwaves to make up a picture of the bile ducts and surrounding organs. You will usually be asked not to eat or drink anything for at least six hours before the scan. Once you are lying comfortably on your back, a gel is spread on your tummy (abdomen). A small device that makes soundwaves is then passed over the area. The soundwaves produce a picture on a computer. The test is painless and only takes a few minutes.

MRI (magnetic resonance imaging) scan

This test uses magnetism to build up a detailed picture of areas of your body. Doctors may use a special type of MRI scan called an MRCP (magnetic resonance cholangiopancreatography). This scan helps the doctors to see the bile ducts.

Before the scan, you may be asked to complete and sign a checklist. This is to make sure it is safe for you to have an MRI scan.

Before having the scan, you will 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 is painless, but having to lie still for a while can be slightly uncomfortable, and some people feel a bit claustrophobic during the scan. It is also noisy, but you will be given earplugs or headphones so you can listen to music. You'll be able to hear and speak to the person operating the scanner.

CT (computerised tomography) scan

A CT scan takes a series of x-rays, which build up a three-dimensional picture of the inside of the body. The scan takes 10–30 minutes and is painless. It uses a small amount of radiation, which is very unlikely to harm you and will not harm anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan.

CT scan
CT scan

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You may be given a drink or injection of a dye, which allows particular areas to be seen more clearly. This may make you feel hot all over for a few minutes. It’s important to let your doctor know if you are allergic to iodine or have asthma, because you could have a more serious reaction to the injection.

You’ll probably be able to go home as soon as the scan is over.

ERCP (endoscopic retrograde cholangio-pancreatography)

This may be used to take an x-ray picture of the bile duct. It can also be used to unblock the bile duct.

You will be asked not to eat or drink anything for about six hours before the test. This is to make sure that your stomach and the first part of your small bowel (the duodenum) are empty. You will be given a tablet or injection to relax you (a sedative). A local anaesthetic spray will be used to numb your throat. Sometimes doctors do this test under general anaesthetic, which means you would be asleep.

The doctor uses a thin, flexible tube called an endoscope. They pass it down your throat into your stomach and the small bowel beyond it. This allows the doctor to look at the bile ducts.

The doctor injects a dye into the bile ducts. This can be seen on x-rays and helps show any abnormalities or blockages. If there is a blockage, your doctor may put in a small tube called a stent to open the duct.

You will be given antibiotics before an ERCP, to help prevent infection. You may need to stay in hospital overnight.

EUS (endoscopic ultrasound scan)

This scan is similar to an ERCP, but the doctor attaches an ultrasound probe to the endoscope. This takes an ultrasound scan of the gall bladder and surrounding areas.

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. You will be given a sedative in the same way as you would before an ERCP.

Your doctor will give you a local anaesthetic injection to numb an area on the right side of your tummy (abdomen). They will then pass a thin needle through the skin into your liver and inject a dye into the bile duct inside the liver. You will have x-rays to see if there is any abnormality or blockage of the bile 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. You will need to stay in hospital for at least one night afterwards.

Biopsy

Your doctor will usually recommend having a biopsy of the tumour. This is to confirm the diagnosis. It involves taking some cells or tissue samples from the affected area of the bile duct. Then they look at the biopsy sample under a microscope. They may take a biopsy during an ERCP or PTC. You may have a CT or ultrasound scan at the same time, to make sure the biopsy is taken from the right place.

If you are having surgery as your main treatment, your doctor may not need to take a biopsy. This might be the case if the scans show it is likely to be cancer.

Laparoscopy

A procedure called a laparoscopy is sometimes used to help diagnose bile duct cancer. It is carried out under a general anaesthetic, so you are not awake. The surgeon uses a tube with a tiny camera attached, called a laparoscope, to look inside your tummy (abdomen).

They make a small cut in your tummy. They then put the laparoscope into the tummy through this cut. This lets them examine the bile duct and the tissue around it for cancer.


Staging and grading of bile duct cancer

Staging

The stage of a cancer describes its size and whether it has spread beyond its original site. Knowing the stage of the cancer helps the doctors plan the best treatment for you.

Cancer can spread through 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 thin tubes. Lymph nodes are small bean-shaped structures. Your doctors will usually examine the lymph nodes close to the bile ducts. This helps them to find out the stage of the cancer.

There are different ways of staging cancers. The TMN staging system is the one usually used for bile duct cancer.

TNM staging system

  • T describes the size of the tumour
  • N describes whether the cancer has spread to the lymph nodes
  • M describes whether the cancer has spread to another part of the body (known as metastatic or secondary cancer).

This system gives detailed information about the tumour stage.

The staging of the cancer is different depending on the part of the bile duct where the cancer started. Your doctor will be able to tell you more about your situation.

Grading

Grading gives an idea of how quickly a cancer may develop. It is based on the way cancer cells look under a microscope:

  • In low-grade tumours, 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. They are likely to grow more quickly and are more likely to spread.


Treatment for bile duct cancer

The treatment you have will depend on:

  • the position of the cancer
  • the size of the cancer
  • whether it has spread beyond the bile duct
  • your general health.

In some situations, the aim of treatment is to relieve symptoms.

Surgery for bile duct cancer

One of the main treatments for bile duct cancer is surgery. Surgery may be done to:

  • try to remove all of the cancer
  • relieve symptoms.

The type of surgery you are offered will depend on:

  • the size of the cancer
  • whether it has begun to spread into nearby tissues
  • your fitness and general health.

Surgery to remove all the cancer

Surgery may be able to cure the cancer for some people. But this is a major operation and is not always suitable. The surgery is complex and is carried out by a surgeon who is a specialist in bile duct cancer. Your surgeon will look at the stage of the cancer to see if this type of operation is possible:


  • If the cancer is at a very early stage – the surgeon will remove only the bile ducts containing the cancer. They then join the remaining ducts in the liver to the small bowel. This allows the bile to flow again.
  • If the cancer is in the bile ducts in the liver (intrahepatic bile duct cancer) – the surgeon will remove the affected part of the liver and the bile ducts.
  • If the cancer is where the left and right hepatic ducts meet just below the liver (perihilar bile duct cancer) – the surgeon will remove the gall bladder and the part of the liver close to the bile ducts. Sometimes the surgeon will also remove part of the pancreas and part of the small bowel.
  • If the cancer is in bile ducts near the pancreas and the small bowel (distal bile duct cancer) – the surgeon will remove the bile ducts, gall bladder, part of the pancreas and part of the small bowel (duodenum). This is sometimes called a Whipple’s operation.

After your operation

You may need to stay in an intensive care ward for the first couple of days. You will then move to a general ward until you recover. Most people need to be in hospital for about 1 to 2 weeks after these types of operation.

Further treatment after surgery to remove the cancer

If it was not possible to remove all the cancer during the operation, you may be offered extra treatment. The aim is to reduce the risk of the cancer coming back. This is called adjuvant treatment. It may involve chemotherapy, radiotherapy or a combination of both, called chemoradiation. Doctors are not sure if adjuvant treatment is effective and these treatments can cause side effects. If your doctor offers you adjuvant treatment, they will talk to you about the possible benefits and disadvantages. This can help you decide whether to go ahead.

Surgery to relieve blockage (obstruction)

If the part of the small bowel called the duodenum is blocked, it can cause sickness (vomiting). This may be helped with an operation to bypass the blockage. The surgeon does this by connecting the stomach to the next section of small bowel (the jejunum).

Sometimes bypass surgery may be done to relieve jaundice caused by a blocked bile duct. But it is more common to have stent insertion. This does not need an operation.

Stent insertion

A stent is a tube that your doctors can put into the bile duct to hold it open. This allows bile to drain away. The tube is thin, and about 5 to 10cm (2 to 4 inches) long. The doctors may put the stent in using an ERCP procedure, or sometimes a PTC procedure:

  • The ERCP method – the preparation and procedure is the same as for ERCP. By looking at x-rays, the doctor can see the narrowing in your bile duct. They stretch the narrowed area using dilators (small, inflatable balloons). They then put the stent in through the endoscope, so the bile can drain. If you have any discomfort while this is being done, it is important to let your doctor know.
  • The PTC method – the procedure and preparation is the same as for PTC. Your doctor passes a wire into the blocked area and then guides the stent along the wire. Sometimes they will put a drainage tube (catheter) in the bile duct. One end of the tube is in the bile duct. The other end stays outside the body, connected to a bag that collects the bile. This can help the doctor insert the stent. They usually leave the tube in for a few days. Once they remove it, the area heals in 2 to 3 days.

Sometimes a combination of ERCP and PTC may be used.

You will be given antibiotics before and after the procedure. This is to prevent infection. You will probably stay in hospital for a few days.

A stent usually needs to be replaced every 3 to 4 months, to stop it becoming blocked. If the stent becomes blocked, you may have a high temperature or jaundice, or both. It is important to tell your specialist as soon as possible if you develop these symptoms. You may need antibiotic treatment. Your specialist may suggest replacing the stent. This is usually straightforward to do.

Chemotherapy for bile duct cancer

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It may be given if you cannot have surgery or if cancer comes back after surgery. The aim is to try to shrink or slow the growth of the cancer and to relieve symptoms. Sometimes chemotherapy may be used after surgery.

Doctors often use a combination of the drugs cisplatin and gemcitabine (Gemzar®) to treat bile duct cancer.

Radiotherapy for bile duct cancer

Radiotherapy treats cancer by using high-energy rays to destroy cancer cells, while doing as little harm as possible to normal cells. It is usually given externally from a machine outside the body. But it can also be given internally by placing radioactive sources inside the body.

Radiotherapy is not often used to treat bile duct cancer. But sometimes it is given on its own or in combination with chemotherapy to:

  • try to lower the risk of cancer coming back after surgery
  • help relieve symptoms.

Doctors are still trying to find out how effective radiotherapy or chemoradiation is in treating bile duct cancer. Your doctor will talk to you about possible benefits and disadvantages if you are offered this treatment.

Selective internal radiotherapy treatment (SIRT)

SIRT is a way of giving internal radiotherapy. It may be used to help relieve symptoms of cancer.

The treatment involves having tiny beads (microspheres) injected into the liver. Each bead is coated with a radioactive substance that gives off radiation. The treatment gives a dose of radiotherapy to the liver over a few days.

The microspheres are harmless and they stay in the liver permanently. The treatment involves staying in hospital for 1 to 4 days.

Side effects include having a high temperature and abdominal pain straight after the injection. These can last for a few days. Other side effects include feeling sick (nausea), being sick (vomiting), and diarrhoea.


Clinical trials for bile duct cancer

Cancer doctors use clinical trials to assess new treatments. If you are asked to take part in a clinical trial, your doctor will discuss the trial with you. This is so you understand what it means to take part.

You may decide not to take part or to withdraw from a trial at any time. If you do, you will then receive the best standard treatment available.


Your feelings about bile duct cancer

Having tests and treatment for cancer can be very stressful. You may have many emotions, including anxiety, anger and fear. These are all normal reactions and part of the process many people go through as they try to come to terms with cancer. We have more information to help you cope with your emotions.

Everyone has their own way of dealing with illness and the different emotions they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse. You can take a look at our Online Community to meet other people in a similar situation. And you can also call our cancer support specialists free on 0808 808 0000.

A UK based charity called AMMF also support people with cholangiocarcinoma.

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