Bile duct cancer

Page last reviewed: 13/07/2011

Cancer of the bile duct is a rare but aggressive type of cancer. Symptoms of bile duct cancer include:

  • jaundice - yellowing of the skin and the whites of the eyes
  • clay-coloured stools (faeces)
  • dark coloured urine

For more information, see Cancer of the bile duct - symptoms.

Cholangiocarcinoma is the medical term for bile duct cancer.

The bile duct

Bile is a fluid that the digestive system uses to help break down fats and digest foods. Bile is also a route through which the body can get rid of toxins. The bile duct system, or 'biliary' system, is made up of a series of tubes that begin in the liver, connect to the gallbladder and end in the small intestine.

The bile ducts help move bile from the liver into the gallbladder before it passes into the small intestine and is eventually passed out.

How common is bile duct cancer?

Bile duct cancer is a rare type of cancer. For every 100,000 people in Ireland, there are one or two new cases of bile duct cancer every year. However, studies suggest that cases of bile duct cancers are increasing in most countries. The reasons for this are unknown.

Who is affected?

Most cases of bile duct cancer occur in people over 70. The condition is slightly more common in men than in women.

Risk factors for bile duct cancer include a rare type of chronic liver disease called primary sclerosing cholangitis, cirrhosis.

However, in most cases there are no obvious reasons why the cancer developed. In parts of the Far East, particularly Thailand, bile duct cancer is approximately 100 times more common than in the West. This is thought to be due to chronic bile duct infection by liver worms, which are common in those areas but not a factor in the West.

Outlook

The outlook for bile duct cancer is poor. The condition can usually only be cured if cancerous cells are limited to the bile duct. If this is the case, some or all of the bile duct can be surgically removed.

However, only 1 in 10 cases of bile duct cancer are diagnosed at this stage and are suitable for surgery. The cancer does not cause symptoms when it is small. Most cases of bile duct cancer are only diagnosed after the cancer has spread beyond the bile duct, by which time a cure is not achievable.

Because of these difficulties, a person diagnosed with bile duct cancer will live for an average of two further years. Only 1 in 20 people will live more than five years after receiving a diagnosis.

Despite this, chemotherapy, endoscopy treatment and surgery can relieve a person's symptoms and improve their quality of life. For more information, see Cancer of the bile duct - Treatment.  

Page last reviewed: 13/07/2011

Cancer of the bile duct does not usually cause any symptoms until it has reached an advanced stage and is blocking the flow of bile from the liver to the small intestine. The blockage will cause bile to move back into the blood and body tissue, resulting in symptoms such as:

  • jaundice- yellowing of the skin and whites of the eyes
  • clay-coloured stools (faeces)
  • dark coloured urine
  • itchy skin
  • weight loss
  • abdominal pain - most people feel a dull ache in the upper right hand side of their abdomen (tummy)
  • high temperature (fever) of 38ºC (100.4ºF) or above
  • chills
  • night sweats

When to seek medical advice

Always visit your GP if you have jaundice. While jaundice is unlikely to be caused by bile duct cancer, it could indicate that there is an underlying problem with the liver, such as hepatitis.

Page last reviewed: 13/07/2011

How cancer begins

Cancer begins with a change in the coding information in cells that tells them when to grow and replicate. The code is read from deoxyribonucleic acid (DNA), which is found in all human cells.

A change in the code is known as a mutation, and it can alter the instructions that control cell growth. The mutation can instruct the cells to carry on growing instead of stopping when they should. This causes the cells to reproduce uncontrollably, resulting in a lump of tissue that is known as a tumour.

How cancer spreads

If left untreated, cancer can quickly grow and spread to other parts of your body.

There are two ways that bile duct cancer can spread:

  • directly - the cancer spreads out of the bile duct and into surrounding tissue and organs, such as the liver, pancreas or gallbladder
  • indirectly - the cancer cells spread via the blood or lymphatic system (see below) to other parts of the body, such as the lungs and bowel

The lymphatic system is a series of glands (or nodes) that are located throughout your body, much like your blood circulation system. The glands produce many of the specialised cells that are needed by the immune system (the body's natural defence against infection and illness).

Risk factors

It is unclear exactly what causes the cells in the bile duct to turn cancerous. However, a number of risk factors have been identified that make it more likely that you will develop the condition. These are discussed below.

Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a rare type of liver disease that causes chronic (long-lasting) inflammation of the liver.

PSC affects about 1 in 16,000 people, and usually occurs in people who are 30-50 years old. An estimated 10-20% of people with PSC will go on to develop bile duct cancer.

Your risk of developing bile duct cancer is thought to be higher if you have PSC and you smoke tobacco.

Viral hepatitis

Hepatitis B and hepatitis C are two types of viral liver infection that are thought to cause a 10-fold increase in the risk of a person developing bile duct cancer.

Studies have found that about 1 in 10 people who develop bile duct cancer test positive for a hepatitis B or hepatitis C infection.

The risk is increased further if a person with hepatitis C has cirrhosis (a scarred liver) as a result of drinking excess amounts of alcohol. In such circumstances, the risk of developing bile duct cancer is thought to be a thousand times higher compared to that of the general population.

Parasitic infection

Liver flukes are a type of parasitic insect that are known to increase the risk of developing bile duct cancer. You can become infected with liver flukes by eating undercooked fish that has been contaminated with fluke eggs.

Liver fluke infections are usually only a problem in Asia (especially Thailand) and Africa, where liver flukes are more widespread.

Billiary stones

Billiary stones are similar to gallstones except that they form inside the liver rather than inside the gallbladder.

Billiary stones are rare in western Europe, but they are relatively common in parts of Asia, such as Japan and Taiwan. It is estimated that approximately 10% of people with billiary stones will develop bile duct cancer.

Exposure to toxins

Exposure to certain chemical toxins is known to increase the risk of developing bile duct cancer.

For example, if you are exposed to a chemical called thorotrast, your chances of developing bile duct cancer rises. Thorotrast was widely used in radiography until it was banned during the 1960s, after its dangerous properties were fully understood.

Other toxins that may increase your chances of developing cancer of the bile duct include:

  • asbestos - a fire-resistant material that was widely used in construction and manufacturing but has now been banned in this country
  • polychlorinated biphenyls (PCBs) - a chemical that was used in manufacturing and building but, like asbestos, has now been banned

Inflammatory bowel disease

Inflammatory bowel disease is a general term that describes a number of conditions that cause inflammation inside the digestive system. The two most common types (although still rare in general terms) of inflammatory bowel disease are:

Ulcerative colitis , and Crohn's disease

People who have either one of these conditions are four times more likely to develop bile duct cancer than the population at large. However, this increased risk is still very small. It is estimated that a person with an inflammatory bowel disease only has a 1 in 1,500 chance of developing bile duct cancer.

Cysts
A cyst is a fluid-filled sac or cavity in the body.
Inflammation
Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.
Liver
The liver is the largest organ in the body. Its main jobs are to secrete bile (to help digestion), detoxify the blood and change food into energy.

Page last reviewed: 13/07/2011

Bile duct cancer can be a challenging condition to diagnose. You usually need to have a number of different tests before an accurate diagnosis can be made. Some possible tests that you may have are described below.

Blood tests

If you have bile duct cancer, the cancerous cells may release certain proteins that can be detected using blood tests. These types of proteins are known as tumour markers.

However, tumour markers can also be caused by other conditions, so a positive blood test does not necessarily mean that you have bile duct cancer.

Scans

A number of scans can be used to examine your bile ducts in more detail and check for lumps or other abnormalities that could be the result of cancer. These scans include:

ultrasound scan  - high frequency sound waves are used to build up a picture of the inside of your body

computer tomography  (CT) scan - a series of X-rays of your liver are taken and a computer is used to assemble them into a more detailed three-dimensional image
magnetic resonance imaging  (MRI) scan - which uses a strong magnetic field and radio waves to produce a picture of the inside of your liver

Endoscopic retrograde cholangiopancreatography (ERCP)

During endoscopic retrograde cholangiopancreatography (ERCP), you will be injected with a special liquid that makes your bile ducts show up more clearly on an X-ray scanner.

The X-ray scanner will be used to guide an endoscope (a small, flexible tube with a camera at the end) down your throat and into your bile duct. The endoscope will be able to detect blockages in your bile duct that could be the result of bile duct cancer.

Percutaneous transhepatic cholangiography (PTC)

Percutaneous transhepatic cholangiography is a procedure carried out to obtain a detailed X-ray image of your bile duct.

The side of your abdomen (tummy) will be numbed using local anaesthetic, and the doctor will pass a needle through your skin and into your liver. A special dye that shows up clearly will be injected into your liver duct.

As with ECRP, PTC is a useful way of detecting any blockages in your bile duct that could be caused by bile duct cancer.

Biopsy

A biopsy is a procedure in which a small sample of tissue is taken from the body and checked under a microscope for the presence of cancerous cells.

A biopsy is usually performed while ERCP or PTC is being carried out. As well as taking bile and tissue samples from your bile duct, samples may be taken from nearby lymph nodes. This is to check whether the cancer has spread from your bile duct and into your lymphatic system.

Staging

After all of the tests listed above have been completed, it should be possible to establish the stage that your cancer is at. The stage describes how far the cancer has spread.

There are two ways of categorising the staging of bile duct cancer. The first is known as the TMN staging system where:

  • T indicates the size of the tumour
  • N indicates whether the cancer has spread to nearby lymph nodes
  • M indicates whether the cancer has spread to other parts of the body (metastasis)

The TMN system is widely used, but it can sometimes be difficult for someone with little or no medical expertise to understand. Therefore, for clarity, the rest of this article will use the second staging system, where the stages of bile duct cancer are described numerically.

The stages are:

  • stage 1A - the cancer is contained inside the bile duct
  • stage 1B - the cancer is beginning to spread beyond the walls of the bile duct but has not spread into the surrounding tissue or lymph nodes
  • stage 2A - the cancer has spread into nearby tissue, such as the liver or pancreas, but has not spread into the lymph nodes
  • stage 2B - the cancer has spread into nearby lymph nodes
  • stage 3 - the cancer has spread into the major blood vessels that supply the lungs, or into organs such as the stomach, gallbladder or bowel
  • stage 4 - the cancer has spread into distant organs, such as the lungs
Biopsy
A biopsy is a test that involves taking a small sample of tissue from the body so it can be examined.
Incision
An incision is a cut made in the body with a surgical instrument during an operation.
MRI
MRI stands for magnetic resonance imaging. It is the use of magnets and radio waves to take detailed pictures of inside the body.
X-ray
An X-ray is a painless way of producing pictures of inside the body using radiation.
Ultrasound scans are a way of producing pictures of inside the body using sound waves.

Coping with the diagnosis

Being diagnosed with cancer, particularly if it is incurable, can be very distressing. For many, the news is difficult to comprehend. Many people who are diagnosed with cancer experience the classic stages of the grieving process. These are outlined below.

  • Denial – you may initially disbelieve the diagnosis and think that there is nothing wrong with you.
  • Anger – you may lash out at friends, family or medical staff.
  • Bargaining – sometimes, people with terminal conditions will try to 'bargain' with their doctors, asking for any sort of treatment that can prolong their life.
  • Depression – you may lose all interest in life and feel that your situation is hopeless.
  • Acceptance – in time, most people eventually accept the diagnosis.

If you have been diagnosed with cancer, talking to a counsellor or psychiatrist may help you to combat feelings of depression and anxiety.

Antidepressants, or medicines that reduce feelings of anxiety, may also be of benefit as you move through the grieving process.

The Irish Cancer Society provides support for people living with cancer.
The Alan Morement Memorial Fund (AMMF) is currently the only dedicated charity on these islands that provides support for people who are affected by bile duct cancer.

The AMMF is a good resource for bile duct cancer. It includes information about the various treatments, plus news about current research into the field.

Page last reviewed: 13/07/2011

Cancer treatment team

Due to the rarity of bile duct cancer, you are likely to be referred to a specialist unit that has experience in treating bile duct cancer, for some or all of your treatment.

Multidisciplinary teams (MDTs) will be used to treat bile duct cancer. MDTs are made up of a number of different specialists (see the box, left). If you have bile duct cancer, you may see some or all of these healthcare professionals as part of your treatment.

It can be difficult to decide what treatment is best for you. Your cancer team will make recommendations, but the final decision will be yours.

Before going to hospital to discuss your treatment options, you may want to write a list of questions to ask the specialist. For example, you may want to find out about the advantages and disadvantages of particular treatments.

Your treatment plan

Your recommended treatment plan will be determined by the stage that the cancer has reached (see Cancer of the bile duct - diagnosis for more information about staging).

In cases of stage one and stage two bile duct cancer, a cure may be possible by surgically removing the affected part of the bile duct, and possibly some of the liver or gallbladder.

In cases of stage three bile duct cancers, the chances of achieving a successful cure will depend on how many lymph nodes have been obstructed. A cure may be possible if only a few nodes have cancerous cells in them, or it may be possible to slow the spread of the cancer by surgically removing the lymph nodes.

In cases of stage four bile duct cancer, achieving a successful cure is highly unlikely. However, chemotherapy, radiotherapy and surgery can often be used to help relieve the symptoms.

A number of experimental treatments may be available as part of a clinical trial (see below for more information).

Surgery

If your MDT think it is possible to cure your bile duct cancer, surgery will be needed to remove any cancerous tissue. Depending on the extent of the cancer, it may be necessary to remove:

  • the part of your bile duct that contains cancerous cells
  • your gallbladder
  • nearby lymph nodes
  • some of your liver
  • In some instances, liver transplantation combined with chemotherapy and radiotherapy may be considered.

Unfortunately, due to the aggressive nature of bile duct cancer, only 1 in 10 people are suitable candidates for surgery.

After surgery, it is usually possible to reconstruct what remains of the bile duct so that bile can still flow normally. Similarly, it is often possible for the liver to resume normal function after surgery because it can regenerate itself.

Having your gallbladder removed should not affect your digestive system because the liver and bile duct can still store bile and aid digestion.

After surgery, it is likely that you will need to stay in an intensive care unit (ICU) for a few days so that the functions of your body can be supported while you recover from the effects of the operation. You may need to stay in hospital for at least two weeks after having bile duct surgery before you are well enough to go home.

The success rates of bile duct surgery depend on a range of individual factors and circumstances, such as whether nearby lymph nodes are free of cancer and whether it was possible to remove all of the cancerous cells during surgery. Both factors will affect the chances of the cancer returning.

As a general estimate, about 40% of people who have surgery for intraheptic bile duct cancer (cancer that begins inside the liver) survive for five years or more after surgery.

20-30% of people who have surgery for extraheptic bile duct cancer (cancer that develops outside the liver) survive for five years or more after surgery.

Unblocking the bile duct

If your bile duct becomes blocked as a result of cancer, your MDT may recommend treatment to unblock the duct. This will help to resolve symptoms such as:

  • jaundice - yellowing of the skin and the whites of the eyes
  • itchy skin
  • abdominal (tummy) pain

Unblocking the bile duct is sometimes necessary if the flow of bile back into your liver starts to affect the normal functioning of your liver.

The bile duct can be unblocked in several ways. The first is to use a variation of the endoscopic retrograde cholangiopancreatography (ERCP) procedure.

During the procedure, a surgeon will guide the endoscope into your bile duct and pass down a small metal or plastic tube called a stent. The stent is used to widen the bile duct, which should help to get the bile flowing again.

Alternatively, a stent can be placed in your bile duct using a variation of the percutaneous transhepatic cholangiography (PTC) procedure. This involves placing the stent in your bile duct through a small incision (cut) in your stomach. As this is not a major operation it can be carried out using local anaesthetic, where an injection is used to numb the skin of your stomach.

Occasionally, an implanted stent can become blocked. If this occurs, it will need to be removed and replaced.

Radiotherapy

Radiotherapy cannot cure bile duct cancer but it can help to relieve the symptoms, slow the spread of the cancer and prolong life. Two types of radiotherapy are used to treat bile duct cancer:

  • external beam radiotherapy - a machine is used to target radioactive beams at your bile duct
  • internal radiotherapy (also known as brachytherapy) - a radioactive wire is placed inside your bile duct next to the tumour

Radiotherapy works by damaging the cancerous cells. However, it can also damage healthy cells and cause side effects. Side effects of radiotherapy include:

  • nausea (feeling sick)
  • vomiting
  • fatigue (severe tiredness)

See the Health A-Z topic about Radiotherapy for more information.

Chemotherapy

Chemotherapy is used in a similar way to radiotherapy to relieve the symptoms of cancer, slow down the rate at which it spreads and prolong life.

Research carried out in 2010 found that combining two chemotherapy medications, called cisplatin and gemcitabine, is a particularly effective way of helping to slow the spread of cancer and improve survival rates.

As with radiotherapy, the medicines that are used in chemotherapy can sometimes damage healthy tissue as well as cancerous tissue, and adverse side effects are common. Side effects of chemotherapy can include:

  • nausea
  • vomiting
  • fatigue
  • hair loss

However, these side effects should stop once the course of treatment has finished. Chemotherapy can also weaken your immune system, making you more vulnerable to infection.

See the Health A-Z topic about Chemotherapy - introduction for more information.

Photodynamic therapy (PDT)

Photodynamic therapy is a new technique that helps to control (but not cure) the symptoms of bile duct cancer. A special chemical is injected into the bile duct, which makes the cancerous cells more sensitive to light. A laser is then passed through an endoscope and used to shrink the tumour.

The National Institute for Clinical Health and Excellence (NICE) has considered PDT and concluded that there is limited evidence to show how effective or safe PDT is in the medium to long term.

If you are considering PDT, be aware of the current uncertainties about the effectiveness and safety of the procedure.

Clinical trials and experimental treatments

The treatments for bile duct cancer are not as effective as treatments for other types of cancer. Therefore, a number of clinical trials are being conducted to find better ways of treating the condition.

For example, ongoing trials are looking at new combinations of chemotherapy medicines and combining chemotherapy with radiotherapy, both of which may help to extend the lifespan of someone with bile duct cancer.

Targeted therapies

Another promising field of research involves using targeted therapies to treat bile duct cancer. Targeted therapies are medication which target the processes that cancerous cells need to grow and reproduce.

As bile duct cancer is a rare condition, there is a possibility you may be invited to take part in a clinical trial that is looking into the use of these types of experimental treatments.

All clinical trials are carried out under strict ethical guidelines that are based on the principles of patient care. However, there is no guarantee that the treatment that you receive during a clinical trial will be more effective, or even as effective, as existing treatments.

Bypass
A bypass is when the flow of blood or other fluid is redirected, permanently because of a blockage in the body, or temporarily during an operation.
Chemotherapy
Chemotherapy is a treatment of an illness or disease with a chemical substance, e.g. in the treatment of cancer. 
Jaundice
Jaundice is a condition that causes yellowing of the skin and the whites of the eyes, brought on by liver problems.
Radiotherapy
Radiation therapy uses x-rays to treat disease, especially cancer.

Your care team

Members of your care team may include:

  • a gastrointestinal surgeon who specialises in treating cancers of the digestive system
  • a clinical oncologist – a specialist in the non-surgical treatment of cancer using techniques such as radiotherapy and chemotherapy
  • a pathologist – a specialist in diseased tissue
  • a radiologist – a specialist in radiotherapy
  • a psychologist
  • a social worker
  • a cancer nurse, who usually acts as the first point of contact between you and the rest of the care team

Content provided by NHS Choices www.nhs.uk and adapted for Ireland by the Health A-Z.

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