Bile duct cancer (cholangiocarcinoma)

Bile duct cancer (cholangiocarcinoma) is almost always a type of cancer called adenocarcinoma and 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 to digest them.

Symptoms of bile duct cancer include:

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

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

The main treatment for bile duct cancer is surgery. You may also have radiotherapy to relieve symptoms. Chemotherapy may also be given before or after surgery, or as the main treatment if you can’t have surgery.

What is bile duct cancer?

Bile duct cancer (cholangiocarcinoma) is almost always a type of cancer called adenocarcinoma. It starts in the lining of the bile duct. Bile duct cancer is rare. Around 1,600 people are diagnosed with it each year in the UK.

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 carry bile. The main function of bile is to break down fats in food to help us to 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 bile ducts connect the liver and gall bladder to the small bowel. When people have had their gall bladder removed, bile flows directly from the liver into the small intestine.

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:

  • intra-hepatic – the bile ducts inside the liver
  • extra-hepatic – the bile ducts outside the liver
  • hilar – this is where the left and right hepatic ducts meet
  • distal – the lower part of the bile ducts, nearest to the bowel.

Bile duct cancer, like other cancers, is not infectious and can't be passed on to other people.


Causes and possible risk factors of bile duct cancer

We don’t yet know what causes most bile duct cancers. But there are a number of risk factors that can increase your risk of developing it. These include:

Inflammatory conditions

People who have a chronic inflammatory bowel condition called ulcerative colitis have a higher risk of developing bile duct cancer.

People who have an inflammatory condition that affects the bile ducts, called primary sclerosing cholangitis, also have a higher risk of developing it.

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.

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.

Age

Although younger people can have bile duct cancers, more than 2 in 3 (67%) occur in people over 65.


Signs and symptoms of bile duct cancer

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. Symptoms of this include:

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

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's important to get them checked by your doctor.


How bile duct cancer is diagnosed

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 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 may be used to diagnose bile duct cancer. You may not have all of them.

Ultrasound scan

This uses soundwaves 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 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.

CT scan

A CT (computerised tomography) scan uses x-rays to build a three-dimensional picture of the inside of the body. You may be given either a drink or injection of dye. This is to make certain areas of the body show up more clearly. We have more detailed information about having a CT scan.

MRI scan

This scan uses magnetism to build up a detailed picture of areas of your body. You may be given an injection of dye, into a vein, to improve the images from the scan. We have more detailed information about having an MRI scan.

ERCP (endoscopic retrograde cholangio-pancreatography)

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

You'll be asked not to eat or drink anything for about six hours before the test, so the stomach and first part of the small bowel (the duodenum) are both empty. You'll be given a tablet or injection to make you relax (a sedative). A local anaesthetic spray will be used to numb your throat. The doctor will then pass a thin, flexible tube called an endoscope through your mouth, into your stomach and into the small bowel just beyond it.

The doctor looks down the endoscope to see the openings where the bile duct and the duct of the pancreas drain into the duodenum. They can inject a dye into these ducts, which can be seen on x-rays, so they can see whether there are any abnormalities or blockages. If there is a blockage, your doctor may insert a small tube known as a stent (see below).

You’ll be given antibiotics beforehand to help prevent any infection and you may need to stay in hospital overnight.

EUS (endoscopic ultrasound scan)

This scan is similar to an ERCP, but an ultrasound probe is attached to the endoscope. This takes an ultrasound scan of the gall bladder 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. 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’ll need to stay in hospital for at least one night afterwards.

Angiogram

This is a test to look at your blood vessels. The bile duct is very close to the large blood vessels that carry blood to and from your liver. An angiogram may be used to check whether any of these blood vessels are affected by the cancer.

Angiograms are done in the x-ray department. A thin tube is put into a blood vessel (artery) in your groin. A dye is then injected up the tube. The dye travels around the arteries so they show up on an x-ray.

Biopsy

The results of the tests listed above may make your doctor suspect you have cancer of the bile duct. But the only way to be sure is by having a biopsy. They take 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.

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 aren't awake.

The surgeon makes a cut (incision) in your tummy to examine the bile duct and the tissue around it for cancer. The surgeon looks inside your tummy using a tube with a tiny camera attached, called a laparoscope.


Staging and grading of bile duct cancer

Staging

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 best treatment for you.

Cancer can spread in the body, either in the bloodstream or through 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. Your doctors will usually examine the lymph nodes close to the biliary system to find out the stage of your 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 anywhere else in the body (secondary cancer or metastases).

This system is complex and it gives precise 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 means the way cancer cells look under a microscope. It gives an idea of how quickly a cancer may develop.

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 and 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

The main treatment for bile duct cancer is surgery to remove the cancer. This may involve a major operation and it isn't always possible. The decision about whether surgery is possible and the type of operation you may have depends on:

  • the size of the cancer
  • 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 bile duct cancer.

Removal of the bile ducts

If the cancer is at a very early stage, the surgeon will remove only the bile ducts containing the cancer. Then they join the remaining ducts in the liver to the small bowel. This allows the bile to flow again.

Partial liver resection

If the cancer has begun to spread into the liver, the surgeon will remove the affected part of the liver and the bile ducts.

Whipple's procedure

If the cancer is larger and has spread into nearby structures, you can have a Whipple’s procedure. This is when the surgeon removes the bile ducts, part of the stomach, part of the small bowel (duodenum), the pancreas, gall bladder and the surrounding lymph nodes.

This is a major operation. After your operation, you may need to 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 relieve jaundice.

The surgeon makes a bypass of the blocked part of the bile duct. This means the bile can flow from the liver into the intestine. Another way to relieve a blockage is to put a tube (stent) into the duct, which holds it open. This can be done without surgery (see below).

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 make a bypass of the blocked duodenum. The surgeon does this by connecting the stomach to the next section of small bowel (the jejunum).

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 about as thick as a ballpoint pen refill and about 5–10cm (2–4in) 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 (see above). By looking at x-rays, the doctor will be able to see the narrowing in your bile duct. They stretch the narrowed area using dilators (small inflatable balloons). Then they put the stent in through the endoscope, so 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 (see above). 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 over in 2–3 days.

Sometimes a combination of ERCP and PTC may be used.

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

A stent usually needs to be replaced every 3–4 months, to stop it 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 they replace the stent. They can usually do this quite easily.

Radiotherapy

Radiotherapy treats cancer by using high-energy rays to destroy cancer cells, while doing as little harm as possible to normal cells. It may occasionally be used to help treat symptoms.

Selective internal radiotherapy treatment (SIRT)

SIRT is a way of giving internal radiotherapy. It may help some people with bile duct cancer. It is usually used to help with symptoms of cancer.

The treatment involves having millions of 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 specifically to the liver over a few days.

The microspheres stay in the liver permanently. They are harmless. The treatment involves staying in hospital for 1–4 days.

Side effects of radiotherapy 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.

Chemotherapy

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 the cancer can't be completely removed. It may also be used if an operation isn't possible or if 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 whether giving chemotherapy after surgery (adjuvant chemotherapy) can help reduce the risk of cancer coming back.

Information and giving consent

Before you go into a trial, a doctor, nurse or other researcher will ask for your permission. They can’t enter you into the trial if you don’t give your written consent, after you have had plenty of time to think about it.

To help you decide whether you want to take part, the researchers should tell you:

  • what the trial is trying to find out
  • what the trial will involve and what you’ll have to do.

There are guidelines for researchers that explain what information people need to help them decide whether to take part in a clinical trial. But there’s a lot of discussion about how much people really want to know, and this varies from person to person.

It’s important that you have enough information to make an informed decision. You should feel able to ask any questions that will help you to make a decision. Before you decide, you should also feel that you have been given enough time to think about the trial and what it will mean to you.

Someone from your medical team will be able to answer any questions you may have. They will go through the possible benefits and risks of joining the trial. They should also discuss any other treatments that may be appropriate in your situation. You may want to talk about it with your family or friends, and think about any practical aspects, such as extra appointments and tests.

You will be given a patient information leaflet about the trial. You can take this away and read it in your own time before you are formally invited to take part.

If you decide to take part

If you decide that you want to take part, you may be asked to give your consent verbally to the person carrying out the trial, who will write it in your notes. You’ll then be asked to sign a consent form that says that you agree to take part. Your doctor will also sign the consent form. You’ll be given a copy to keep.

If you decide not to take part

If you decide not to take part in the trial, you can tell your doctor or nurse. Your decision will be respected and you don’t have to give a reason. There will be no change in the way that the hospital staff treat you, and you’ll be offered the standard treatment for your type of cancer.


Your feelings

Having tests and treatment for cancer can be very stressful. You may have many different emotions, including anxiety, anger and fear. These are all normal reactions that many people go through when dealing with cancer.

Everyone has their own way of coping with difficult situations. Some people find it helpful to talk to family or friends, while others prefer to seek help from people outside their situation. Some people prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is there if you need it. Our cancer support specialists can give you information about counselling in your area.

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