Bile duct cancer

Bile duct cancer is a rare but aggressive type of cancer.

Introduction

Bile duct cancer is a rare but aggressive type of cancer.

Cholangiocarcinoma is the medical term sometimes used for bile duct cancer.

In most cases, there are no signs of bile duct cancer until it reaches the later stages, when symptoms can include:

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

See your doctor if you have signs of jaundice or are worried about other symptoms. While it is unlikely you will have bile duct cancer, it is best to get it checked.

Read more about the symptoms of bile duct cancer.

Why does bile duct cancer happen?

The exact cause of bile duct cancer is unknown. However, there are some things that can increase your chances of developing the condition. These include a chronic liver disease called primary sclerosing cholangitis (PSC), hepatitis B and hepatitis C.

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.

Read more about the causes of bile duct cancer.

Types of bile duct cancer

There are two main types of bile duct cancer, depending on where the cancer begins:

  • cancer that starts in a part of the bile duct that is inside the liver is known as intrahepatic bile duct cancer
  • cancer that starts in part of the bile duct outside the liver is known as extrahepatic bile duct cancer

Usually, intrahepatic bile duct cancer is treated in a similar way to primary liver cancer.

Diagnosis

Cancer of the bile duct can be difficult to diagnose, so you may need several tests, including blood tests, ultrasound scans, computerised tomography (CT) scans, and magnetic resonance imaging (MRI) scans. Some of these tests may require you to be injected with a special dye that highlights your bile ducts so they are easier to examine.

If possible, you will probably need a biopsy before bile duct cancer can be diagnosed. This involves removing a small sample of tissue so it can be studied under a microscope.

However, in some cases your surgeon may prefer to remove the suspected tumour based on the results of your scans alone.

Read more about diagnosing bile duct cancer.

How is bile duct cancer treated?

Cancer of the bile duct can usually only be cured if cancerous cells haven't spread. If this is the case, some or all of the bile duct may be surgically removed.

Unfortunately, only a small proportion of bile duct cancer cases are diagnosed at this stage and are suitable for surgery. This is because signs and symptoms usually develop at a late stage.

Despite this, radiotherapy, chemotherapy and, to a lesser extent, surgery can relieve the symptoms of bile duct cancer and improve the quality of life of people in the advanced stages of the condition.

Read more about treating bile duct cancer.

Who is affected?

Bile duct cancer is rare. Only about 1,000 people are diagnosed with the condition each year in the UK.

However, studies suggest that cases of bile duct cancer are increasing in most countries. The reasons for this are unknown.

Most cases of bile duct cancer occur in people over 60. The condition affects men and women almost equally.

Can bile duct cancer be prevented?

There are no guaranteed ways to avoid getting bile duct cancer. However, it is possible to reduce your chances of developing the condition.

The most effective ways of achieving this are not smoking, reducing your alcohol intake, and trying to ensure you do not become infected with hepatitis B or hepatitis C.

Read more about preventing bile duct cancer.

Symptoms

Bile duct cancer does not usually cause any symptoms until the flow of bile from the liver is blocked.

In most cases, the condition is at an advanced stage by this time.

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
  • fatigue
  • loss of appetite
  • 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 doctor 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.

Causes

The exact cause of bile duct cancer is unknown, although there are some things that can increase the risk of developing the condition.

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), found in all human cells.

A change in the code is known as a mutation, and 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 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 and its blood vessels, the pancreas or the 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) located throughout your body, much like your blood circulation system. The glands produce many of the specialised cells needed by the immune system (the body's natural defence against infection and illness).

Increased risk

A number of things have been identified that make it more likely that you will develop the condition. Some of these are discussed below.

Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a rare type of liver disease that causes long-lasting (chronic) inflammation of the liver. It usually occurs in people who are between the ages of 30 and 50. In most cases, PSC is associated with chronic inflammation of the colon (ulcerative colitis).

It is not known exactly how many people with PSC will develop bile duct cancer, but estimates vary from 5-35%.

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

Bile duct abnormalities

Some people can have fluid-filled sacs (cysts) in their bile duct. These cysts are usually congenital, which means they are present from birth.

The most common types are choledochal cysts and Caroli's disease.

It is estimated that 6-30% of people with these conditions will develop cancer of the bile duct.

Biliary stones

Biliary stones are similar to gallstones, except they form inside the liver rather than inside the gallbladder.

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

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
  • Crohn's disease

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

Viral hepatitis

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

Studies have found that about one 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 these circumstances, the risk of developing bile duct cancer is thought to be 1,000 times higher compared to that of the general population.

Parasitic infection

Liver flukes are a type of parasitic insect 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.

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 which was widely used in construction and manufacturing but is now banned in this country
  • polychlorinated biphenyls (PCBs) – a chemical that was used in manufacturing and building but, like asbestos, has now been banned

Other factors

Diagnosis

Bile duct cancer can be a challenging condition to diagnose. You usually need several different tests before an accurate diagnosis can be made.

You may visit your doctor first, who will examine you and ask about your symptoms and medical history. If cancer is suspected, you will be referred to a specialist for tests.

Some of these are described below.

Blood tests

If you have bile duct cancer, the cancerous cells may release certain chemicals 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. A positive blood test does not necessarily mean you have bile duct cancer, and a negative blood test does not always mean you don't.

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 – this 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 is 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 can detect blockages in your bile duct that could be the result of bile duct cancer.

Spyglass

An advanced form of ERCP is a special test called a spyglass. This involves passing a specialised endoscope into the bile duct so any abnormalities can be spotted. It also enables a biopsy to be taken at this stage.

While this test can help confirm a diagnosis in uncertain cases, it is very expensive and requires specialist training. Therefore, it is only available at a limited number of centres.

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) is numbed using local anaesthetic, and a special dye that shows up on X-rays is injected through your skin and 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

If scans and tests indicate you may have bile duct cancer, a biopsy is carried out to confirm a diagnosis.

During a biopsy 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 into your lymphatic system.

Staging

If tests confirm you have cancer, it should be possible to establish the stage 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 TNM 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 TNM system is widely used, but can sometimes be difficult for non-medical professionals to understand. Therefore, this article uses 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 tissue and 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

Treatment

Most cases of bile duct cancer cannot be cured. Instead, treatment is most commonly used to relieve symptoms.

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 the condition for some or all of your treatment.

A multidisciplinary team (MDT) made up of different specialists will help you decide on your treatment. The 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 your general health and the stage the cancer has reached.

In cases of stage 1 and stage 2 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 3 bile duct cancers, the chances of achieving a successful cure will depend on how many lymph nodes have been affected. 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 4 bile duct cancer, achieving a successful cure is highly unlikely. However, chemotherapy, radiotherapy and surgery can often be used to help relieve the symptoms.

Read about diagnosing cancer of the bile duct for more information about staging.

Your treatment plan may also be different if you have intrahepatic bile duct cancer, as this is usually treated in a similar way to liver cancer.

Read more about treating liver cancer.

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

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

Unfortunately, due to the aggressive nature of bile duct cancer, fewer than one-third of patients are suitable for surgery.

After surgery, it is usually possible to reconstruct what remains of the bile duct so that bile can still flow into the intestine.

Similarly, it is often possible for the liver to resume normal function after surgery because we do not need all of our liver. For example, 25-30% of an otherwise healthy liver is enough for you to survive. The liver can also regenerate itself to some extent after surgery.

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 you will need to stay in an intensive care unit (ICU) for a few days so 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 two weeks or more after having bile duct surgery before you are well enough to go home.

Success rates of bile duct surgery depend on 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.

In addition, the underlying cause of the cancer may increase the risk of the condition returning.

As a general estimate, 10-40% of people who have surgery for bile duct cancer survive for five years or more after their operation.

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 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 a long, flexible tube with a light and video camera on the end (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 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 (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)

Read more about radiotherapy.

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. It is sometimes used in combination with radiotherapy.

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, medicines 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.

Read more about chemotherapy.

Photodynamic therapy (PDT)

Photodynamic therapy is a new technique that can help 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 Health and Care Excellence (NICE) has considered PDT and concluded there is limited evidence to show how effective or safe it is in the medium to long term.

If you are considering PDT, it is important to 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, which may help 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 medications that target the processes that cancerous cells need to grow and reproduce.

In studies, a medication called sorafenib has proved reasonably effective. Sorafenib works by blocking a protein that cancerous cells need to create a blood supply. However, sorafenib is not currently used as routine treatment for cancer of the bile duct.

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

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

Read more about [clinical trials].

Prevention

There are no guaranteed ways to avoid getting bile duct cancer, although it is possible to reduce your chances of developing the condition.

The three most effective steps to reduce your chances of developing bile duct cancer are:

  • giving up smoking (if you smoke)
  • drinking alcohol in moderation
  • minimising your exposure to the hepatitis B and hepatitis C viruses

Stopping smoking

Not smoking is the most effective way of preventing bile duct cancer, as well as other serious health conditions, such as stroke, heart attack and lung cancer.

It is particularly important to stop smoking if you have the liver condition known as primary sclerosing cholangitis (PSC). If you have PSC, smoking will significantly increase your chances of developing bile duct cancer.

Your doctor can advise on how to give up smoking. They can also recommend and prescribe suitable medication.

Alcohol

If you are a heavy drinker, reducing your alcohol intake will help prevent liver damage (cirrhosis). This may in turn reduce your risk of developing bile duct cancer. Reducing your alcohol consumption is particularly important if you have a pre-existing liver condition, such as PSC or hepatitis B or C.

The recommended daily levels of alcohol consumption are:

  • 3-4 units of alcohol for men
  • 2-3 units of alcohol for women

A unit of alcohol is equal to about half a pint of normal-strength lager, a small glass of wine or a pub measure (25ml) of spirits.

Visit your doctor if you are finding it difficult to moderate your alcohol consumption. Counselling and medication are available to help reduce the amount you drink.

Hepatitis C

Those most at risk of getting a hepatitis C infection are people who regularly inject illegal drugs, such as heroin.

If you regularly inject drugs, the best way to avoid getting hepatitis C is to never share any of your drug-injecting equipment with others. This does not just apply to needles but anything that could come into contact with other people's blood, such as:

  • mixing spoons
  • filters
  • water used to dissolve drugs
  • tourniquets (the belt sometimes tied around the arm to make the veins easier to inject)

Hepatitis C does not cause any noticeable symptoms for many years, so people may be unaware they are infected. It is therefore safer to assume anyone may have the infection.

It may also be possible to get hepatitis C by sharing banknotes or "snorting tubes" with an infected person to snort drugs, such as cocaine or amphetamine. These types of drugs can irritate the lining of your nose and small particles of contaminated blood could be passed onto the note or tube before being inhaled.

Even if you are not a drug user, you can take commonsense precautions to minimise your exposure to other people's blood. Avoid sharing any object that could be contaminated with blood, such as razors and toothbrushes.

There is less risk of getting hepatitis C by having sex with someone who is infected. However, as a precaution, it is best to use a barrier method of contraception, such as a condom.

Read more about getting tested and treated for hepatitis C.

Hepatitis B

A vaccine is available that provides immunisation against hepatitis B.

Vaccination is usually only recommended for people in high-risk groups, such as:

  • injecting drug users (including their partners and children and other people living with them)
  • people who change sexual partners frequently (including men who have sex with men, and male and female sex workers)
  • close family contacts of someone with a chronic (long-term) hepatitis B infection
  • people who receive regular blood products and their carers
  • people who have chronic kidney failure
  • people who have chronic liver disease
  • prisoners and some prison service staff
  • people who live in residential accommodation for those with learning difficulties
  • families that foster or adopt children who may have been at increased risk of developing a hepatitis B infection
  • people travelling to, or going to live in, areas where there is a high or moderate incidence of hepatitis B, such as China

You should also be vaccinated if you have a job that increases your exposure to hepatitis B. At-risk occupations include:

  • healthcare workers
  • laboratory staff
  • staff who work in residential care homes for those with learning difficulties
  • morticians and embalmers
  • some emergency services personnel

Visit your doctor for advice if you are uncertain about whether you should be vaccinated against hepatitis B.

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