Bile duct cancer (cholangiocarcinoma)

Bile duct cancer (cholangiocarcinoma) is a rare but aggressive type of cancer.

The bile duct system, or 'biliary' system, is made up of a series of tubes that begin in the liver and end in the small intestine. Bile is a fluid the digestive system uses to help break down fats and digest foods.

Symptoms of 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, itchy skin, pale stools and dark-coloured urine 
• unintentional weight loss
• abdominal pain

Speak to your GP if you have signs of jaundice or are worried about other symptoms. While it is unlikely you 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, some things may increase your chances of developing the condition. The most common include being over 65 years old or having a rare chronic liver disease called primary sclerosing cholangitis (PSC).

Read more about the causes of bile duct cancer

Types of bile duct cancer

There are 2 main types of bile duct cancer, depending on where the cancer begins:

• cancer that starts in a part of the bile duct 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

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
• magnetic resonance imaging (MRI) scans

For some of these tests, you may need to be injected with a special dye that highlights your bile ducts.

You may also need a biopsy. 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 removed.

Only a small proportion of bile duct cancer cases are diagnosed early enough to be suitable for surgery. This is because symptoms usually develop at a late stage.

Despite this, treatment such as chemotherapy 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?

Most cases of bile duct cancer occur in people over the age of 65. 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, but you can reduce your chances of developing it.

The most effective ways of achieving this are reducing your alcohol intake, as cirrhosis is a risk factor, and trying to ensure that you don't become infected with hepatitis B or hepatitis C.

Read more about preventing bile duct cancer

Bile duct cancer doesn't 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, itchy skin, pale stools and dark-coloured urine
• unintentional weight loss 
• abdominal pain – most people feel a dull ache in the upper right-hand side of their abdomen (stomach)
• high temperature (fever) of 38C (100.4F) or above and shivering
• loss of appetite

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 an underlying problem with the liver, such as hepatitis.

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

Cancer begins with a change (mutation) in the structure of the DNA in cells, which can affect how they grow. This means that cells grow and reproduce uncontrollably, producing a lump of tissue called a tumour.

If left untreated, cancer can grow and spread to other parts of your body, either directly or through the blood and lymphatic system.

Increased risk

A number of factors that increase the risk of developing bile duct cancer have been identified. 

Age

Your chances of developing cancer of the bile duct increase as you get older. Most people with the condition are over 65 years old.

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 aged 30 to 50. Up to 10% of people with ulcerative colitis (inflammation of the colon and rectum) also have PSC.

Around 10 to 20% of people with PSC will develop bile duct cancer. 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, but both conditions are very rare.

Up to 20% of people with choledochal cysts that are not removed 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's estimated that approximately 10% of people with biliary stones will develop bile duct cancer.

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 that 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

The following factors are linked with an increased risk of developing bile duct cancer, but more research is still needed:

• hepatitis B and hepatitis C
• cirrhosis (a scarred liver) as a result of drinking excessive amounts of alcohol
• HIV
• diabetes
• obesity
• smoking

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

Blood tests

In bile duct cancer, the cancerous cells may release certain chemicals that can be detected using blood tests. These 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 cholangio-pancreatography (ERCP)

Endoscopic retrograde cholangio-pancreatography (ERCP) allows your bile ducts show up more clearly on an X-ray scanner.  

A special dye is injected and 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.

A short wire-mesh tube, called a stent, may also be inserted into the artery during the procedure. The stent is left in place permanently to allow blood to flow more freely.

Spyglass

An advanced form of ERCP is a special test called a spyglass. This involves passing a specialised endoscope into the bile duct to detect abnormalities. It also enables a biopsy to be taken.

While this test can help confirm bile duct cancer 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 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, a stent may be inserted during the procedure. PTC and ECRP are both useful ways of detecting any blockages in your bile duct that could be caused by bile duct cancer.

Biopsy

If scans indicate that you may have bile duct cancer, a biopsy may be 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 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

A widely used method of staging bile duct cancer is a number staging system. 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 2 – the cancer has spread into nearby tissue, such as the liver, but has not spread into the lymph nodes
• stage 3 – the cancer has spread into the lymph nodes and major blood vessels that supply the liver
• stage 4 – the cancer has spread into distant organs, such as the lungs

Read more about staging bile duct cancer on the Cancer Research UK website

Cancer support

If you are diagnosed with cancer, the Alan Morement Memorial Fund (AMMF) is currently the only dedicated UK charity that provides support for people affected by bile duct cancer.

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 hepatobiliary unit with experience in treating the condition.

A multidisciplinary team (MDT) made up of different specialists will help you decide on your treatment, but the final decision will be yours. Your MDT may include:

• a liver surgeon – a specialist in treating cancers of the liver
• a medical or 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 radiological diagnosis and intervention 
• a cancer nurse – who usually acts as the first point of contact between you and the rest of the care team 
• a gastroenterologist – a specialist in the medical management of liver and biliary disease

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 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, stenting, chemotherapy, radiotherapy and surgery can often be used to help relieve the symptoms.

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.

Surgery

If your MDT thinks 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
• a large part of your liver

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. The liver can also regenerate itself after surgery.

You may need to stay in hospital for up to two weeks or more after having bile duct cancer surgery before you are well enough to go home.

Success rates of bile duct surgery depend on individual 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.

Unblocking the bile duct

If your bile duct becomes blocked as a result of cancer, treatment to unblock it may be recommended. 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 by using a small tube called a stent. The stent widens the bile duct, which should help to get the bile flowing again.

A stent can be inserted using either:

• a variation of the endoscopic retrograde cholangiopancreatography (ERCP) procedure, which uses an endoscope to guide the stent into the bile duct
• a variation of the percutaneous transhepatic cholangiography (PTC) procedure, which involves making a small incision (under local anaesthetic) in your abdominal wall

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

Radiotherapy

Radiotherapy is not a standard treatment for bile duct cancer, but may help to relieve the symptoms, slow the spread of the cancer and prolong life. The 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 it spreads and prolong life. It is sometimes used in combination 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

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 for some cancers, 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 a routine treatment for bile duct cancer.

As bile duct cancer is a rare condition, there is a possibility you may be invited to take part in a clinical trial looking at 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 that the treatment you receive during a clinical trial will be more effective, or even as effective, as existing treatments.

Read more about clinical trials and clinical trials for bile duct cancer

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

The 3 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 GP can advise on how to give up smoking. They can also recommend and prescribe suitable medication. You can get more information and advice from the Quit Your Way Scotland advice and support service.

Read more about stopping smoking

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.

Read low-risk drinking guidelines

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

Read more about alcohol

Hepatitis C

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.

You should also 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, with a new partner.

Read more about preventing hepatitis C

Hepatitis B

A vaccine is available that provides immunisation against hepatitis B. 

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

• people who inject drugs or have a sexual partner who injects drugs
• people who change their sexual partner frequently
• people travelling to or from a part of the world where hepatitis B is widespread
• healthcare workers who may have come into contact with the virus

Pregnant women are also screened for hepatitis B. If they are infected, their baby can be vaccinated shortly after birth to prevent them also becoming infected.

Read more detailed information about hepatitis B vaccination

The liver fluke

The liver fluke is a major cause of bile duct cancer in Asia. It is a parasite which, after infection, damages the tissue of the bile duct and in some cases triggers the onset of bile duct cancer.

The liver fluke is widespread in Thailand. Other countries where the liver fluke can be found include:

• Cambodia
• Laos
• Vietnam

Infection occurs after eating raw or undercooked fish contaminated by fluke eggs.

Always ensure any fish you eat is cooked thoroughly when travelling in these countries.