Bile duct cancer (cholangiocarcinoma)

Bile duct cancer, also called cholangiocarcinoma, is a rare cancer. It starts in the lining of the bile duct.

Bile ducts run both inside and outside the liver. Most cases of bile duct cancer (cholangiocarcinoma) start in the lining of these ducts.

There is more than one type, and each is named after the part of the bile duct where the cancer began.

Intrahepatic bile ducts are the bile ducts inside the liver. Cancers that start in them, called intrahepatic bile duct cancers, make up 1 to 2 in every 10 bile duct cancers (10 to 20%).

Extrahepatic bile ducts are the bile ducts outside the liver. Cancer that starts in them is called extrahepatic bile duct cancer (a type of cholangiocarcinoma), and there are 2 types:

• perihilar bile duct cancers, which make up 5 in 10 bile duct cancers (50%) and start in the area where the bile ducts leave the liver
• distal bile duct cancers, which make up 3 to 4 in 10 bile duct cancers (30 to 40%) and start lower down, nearer the small bowel

Bile duct cancer (cholangiocarcinoma) doesn't usually cause symptoms in its early stages. When symptoms do appear, they're often vague and can include:

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

But many different things can cause these symptoms.

Symptoms of bile duct cancer may occur if the cancer blocks the bile duct. This stops bile flowing between the liver and the bowel, so the bile flows back into the blood and body tissues. This can cause jaundice.

Symptoms of jaundice include:

• yellowing of the skin and eyes
• dark-yellow pee (urine)
• pale, loose poo (stool) that's difficult to flush
• itchy skin

If you're worried about any symptoms, get them checked by your doctor.

Doctors don't know exactly what causes bile duct cancer. There are, though, some risk factors that can raise your chance of developing it.

If you have symptoms of bile duct cancer (cholangiocarcinoma), you'll usually start by seeing your doctor. They'll examine you and may do some blood tests. They may also arrange further tests and refer you to a specialist at the hospital.

At the hospital, you may see a liver specialist (hepatologist). Or you may see a doctor who specialises in treating conditions of the stomach and bowel (gastroenterologist).

The doctor will ask you about your symptoms and any previous medical problems. They'll also examine you and take blood samples. This is to check:

• your general health
• how well your liver is working

There are many different tests for bile duct cancer. Some of these are described here. You may have one or more of them.

An external ultrasound scan uses sound waves to build up a picture of the bile ducts and the organs around them.

A CT scan uses a series of X-rays to build up a three-dimensional (3D) picture of the inside of your body.

An MRI scan uses magnetism to build a detailed picture of areas inside your body.

An MRCP (magnetic resonance cholangiopancreatography) is a type of MRI scan that looks at the bile ducts and nearby organs. Your doctor can tell you more about this test.

An ERCP (endoscopic retrograde cholangio-pancreatography) lets the doctor look at your bile ducts, gallbladder and pancreas. During the test, the doctor can take small samples of tissue (biopsies). They can also treat a blockage in the bile ducts, which helps with jaundice.

An EUS is much like an ERCP, but it uses ultrasound. It can also be used to take biopsies.

If you have bile duct cancer (cholangiocarcinoma), the doctors will take a sample of the cancer cells if they can. This is called a biopsy. They may do it during a test like an ERCP or EUS, and a biopsy can help confirm a diagnosis.

The cancer cells can also be tested in a laboratory, to see whether there are any changes to certain genes. This is called molecular testing.

If the cancer cells have a change in a particular gene, you may be offered a drug that targets it. This is called precision medicine.

Macmillan Cancer Support have more information about genes and cancer treatment. The charity AMMF has information about bile duct cancer and molecular testing.

A team of specialists will meet to discuss the best treatment for you. This is called a multidisciplinary team (MDT). Bile duct cancer is a hepatobiliary cancer, so you'll usually be seen by an MDT that looks after these types of cancers.

After the MDT meeting, you'll usually meet your specialist doctor and nurse. They'll talk to you about your treatment plan.

The treatment you have depends on:

• the type of bile duct cancer you have
• the size of the cancer
• whether the cancer has spread to other areas of the body
• your general health
• your preferences

Your specialist doctor should explain:

• the aim of the treatment, whether it's to cure the cancer or control it
• the benefits of the treatment
• the disadvantages of the treatment, like the risks and side effects
• any other treatments that may be available
• what may happen if you don't have the treatment

Your doctor or cancer specialist or nurse will explain the different treatments and their side effects. They'll also talk to you about things to consider when making treatment decisions. Make sure you have a contact number so you know who to call if you have further questions.

When doctors plan treatment for bile duct cancer (cholangiocarcinoma), they often describe the tumour as resectable or unresectable.

A resectable tumour is one that doctors think may be possible to remove with surgery. Early-stage cancers (stage 1 or 2) are often resectable, and some stage 3 cancers may be too.

An unresectable tumour can't be removed with surgery. Sometimes this is because the cancer has spread to parts of the body far from the bile ducts. Or the cancer may be affecting structures that can't be removed, like important blood vessels. Unresectable tumours include some stage 3 cancers and stage 4 cancers.

To decide whether a cancer can be removed safely (whether it is resectable), your doctor needs to consider where the cancer is and how well you are in general.

Some people with bile duct cancer (cholangiocarcinoma) may be offered treatment that tries to cure the cancer. But this is only possible for a small number of people.

If the cancer can't be cured, you can still have treatment to control its growth and help with symptoms. There are different treatments that can be used.

Your cancer doctor or nurse will talk to you about your treatment options:

Some people with bile duct cancer (cholangiocarcinoma) can have an operation to remove the cancer, and this can be done to try to cure it.

Surgery is also sometimes used to relieve symptoms when the cancer is blocking the bowel or the bile ducts.

Bile duct cancer (cholangiocarcinoma) often blocks the bile ducts, and that can cause jaundice, which is a yellowing of the skin and eyes.

Doctors usually treat the blockage by putting a tube called a stent into the blocked bile duct.

Sometimes the duodenum, which is a part of the small bowel, gets blocked. The stomach normally empties into the duodenum, so a blockage there can make you vomit. A stent put into the blocked area can help. It lets food pass through and eases the sickness.

The doctor puts the stent in using an endoscope, which is a long, flexible tube with a camera on the end. If a stent can't be put in, you may be offered an operation to bypass the blockage. The surgeon joins the stomach to the next section of small bowel (the jejunum), so the blocked part is avoided.

If you have bile duct cancer (cholangiocarcinoma), you'll usually have chemotherapy after surgery. It's given to help lower the risk of the cancer coming back.

If surgery isn't possible, your doctor may offer chemotherapy to shrink the cancer or slow its growth.

After surgery for bile duct cancer (cholangiocarcinoma), you may be given radiotherapy to lower the risk of the cancer coming back. It can be given on its own or together with chemotherapy.

If the cancer has not spread outside of the liver, a type called stereotactic ablative radiotherapy (SABR) may be used.

Radiotherapy is also sometimes used to treat the symptoms that bile duct cancer causes.

Targeted therapy drugs work on something in or around the cancer cell that helps it grow and survive. Doctors may use them to treat locally advanced or advanced bile duct cancer (cholangiocarcinoma).

Immunotherapy for bile duct cancer (cholangiocarcinoma) uses drugs that work with your immune system, helping it find and attack cancer cells. Doctors may use it when the cancer:

• isn't suitable for surgery (unresectable)
• has spread to other parts of the body

You may have some treatments as part of a clinical trial.

Even if you already live a healthy life, you may still want to make a few positive changes after treatment for bile duct cancer (cholangiocarcinoma). Small things like eating well and staying active can improve your health and wellbeing, and they help your body recover.

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