Cholangiocarcinoma (bile duct cancer)

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Key points

Cholangiocarcinoma is a rare cancer that grows in the bile ducts
It’s hard to treat as you might not know you have it until it’s spread
Treatment may involve surgery, chemotherapy, or a combination of chemotherapy and a different type of medicine called immunotherapy
It’s important to make sure you have a nutritious diet if you have cholangiocarcinoma

What is cholangiocarcinoma?

Cholangiocarcinoma is a cancer that grows in the bile ducts. The bile ducts are tubes that carry bile from the liver to the gallbladder and then to the small intestine. Cholangiocarcinoma is sometimes just called bile duct cancer.

There are different types of cholangiocarcinoma, depending where in the bile duct the cancer grows.

Intrahepatic cholangiocarcinoma is when the cancer grows in the part of the bile duct that is inside the liver itself.
Hilar cholangiocarcinoma is when the cancer grows in the bile ducts just outside of the liver. Sometimes this type of cancer is called perihilar cholangiocarcinoma.
Distal cholangiocarcinoma is when the cancer grows in the part of the bile duct near to the small intestine. Sometimes this type of cancer is called extrahepatic cholangiocarcinoma.

Cholangiocarcinoma is a rare cancer. Only about 1 in every 10 primary liver cancers grows in the bile ducts.

What are the symptoms of cholangiocarcinoma?

Most people don’t realise they have cholangiocarcinoma because it doesn’t cause any symptoms at first.

Sometimes people may notice symptoms including:

Chills and/or fever, night sweats
Light-coloured poo
Dark urine
Feeling very tired and weak
Being very itchy (also called pruritus)
Losing your appetite
Losing weight without trying to
Feeling nauseous
Pain in the upper right belly that may radiate to the back
Yellow skin and eyes (jaundice)

Let your doctor know if you notice any of these symptoms and they don’t go away.

Symptoms of liver disease

How is cholangiocarcinoma diagnosed?

Most people with cholangiocarcinoma first see their doctor for symptoms such as abdominal pain, weight loss or abnormal blood tests. First your doctor will probably order some scans. You might have an ultrasound, CT scan or MRI. Some people have a special type of MRI called magnetic resonance cholangiopancreatography (MRCP) that shows up the bile ducts in more detail.

Your doctor will probably order a blood test to look for signs of cancer in your blood. One of the signs you may have cholangiocarcinoma is raised levels of a protein called CA 19-9. The blood test will also look at how well your liver is functioning.

If a lump is seen in the liver, your doctor may take a small sample of tissue (biopsy) to look at in the laboratory.

Another way of checking your bile ducts is through a procedure called endoscopic retrograde cholangiopancreatography (ERCP). In this procedure, an endoscope (a flexible tube with a light and camera) is inserted through your mouth into your stomach and then into the upper small bowel. A thin tube (called a catheter) is passed through the endoscope into the bile duct. Dye is injected through the catheter while X-rays are taken. If it’s necessary and possible, a biopsy may be taken, and a tube called a stent may be passed into the bile ducts to help bile flow past any blockage.

Liver tests

Why do people get cholangiocarcinoma?

The main reason people develop cholangiocarcinoma is because they have had inflammation in their bile ducts for a long time. This might be caused by other long-lasting liver conditions like hepatitis, fatty liver disease or primary sclerosing cholangitis (this can cause scarring in the bile ducts).

You are more at risk of developing cholangiocarcinoma if:

You have inflammatory bowel disease or diabetes
You were born with problems in the bile ducts or other genetic conditions including cystic fibrosis or Lynch syndrome
You have been exposed to certain chemicals (especially chemicals used in the printing industry)
You have a parasite called liver fluke, which you can get from eating raw or undercooked fish in parts of Southeast Asia
You’re a smoker

Cholangiocarcinoma is more common in people over the age of 50.

How is cholangiocarcinoma treated?

If you have cholangiocarcinoma, you will be referred to a specialist such as a liver surgeon, gastroenterologist or medical oncologist. Your case will likely be reviewed by a team of specialists at a multidisciplinary team meeting.

The first job is to ‘stage’ the cancer. This means your medical team will work out the size of the tumour, whether any lymph nodes are affected, and how far it’s spread. You will get a score with numbers from 1 to 4. The higher the numbers, the more advanced the cancer is.

Possible treatments depend on your unique tumour and how widespread it is. You might have:

Surgery to remove part of the liver or insert a stent into the bile duct to help the bile flow past a blockage
Chemotherapy with or without immunotherapy to kill the cancer cells (given via a drip in your vein). Immunotherapy is a new way of treating cholangiocarcinoma by boosting your own immune system to attack the cancer.
Radiation therapy to reduce the size of the cancer or treat spots where the cancer has spread
Liver transplant may very rarely be considered for treatment of small cancers in the liver

Cholangiocarcinoma can be quite difficult to treat successfully because usually it’s already spread by the time people are diagnosed.

Treatments

Living with cholangiocarcinoma

If you are diagnosed with cholangiocarcinoma, there are some important things you can do to make sure you’re as healthy as you can be.

Treatment for cholangiocarcinoma might make you tired and sick. Any problems with the bile ducts can affect your digestion and you might have diarrhoea, constipation or mouth ulcers. You might just not feel hungry.

Good nutrition will help you keep a healthy weight, maintain your muscle strength and give you energy.

Here are some nutrition tips for people with cholangiocarcinoma:

Talk to your doctor or an accredited practising dietitian about a suitable diet that packs as much nutrition as possible into everything you eat
You might need to eat more kilojoules than you’re used to – it’s important to keep up your weight
Eat a small nutritious snack every 2-3 hours
Don’t worry about low fat foods – eat full fat options
Replace tea and coffee with soups, milks, milkshakes or smoothies
Do some light exercise, like walking, to improve your appetite and help your digestion
Consider nutritional supplements – but talk to your doctor or dietitian first

Diet and nutrition

Some other ways to look after yourself are:

Avoid all alcohol to protect your liver
Avoid anything that is toxic to your liver, including some medications, alternative medicines and liver tonics
Look after any other health conditions you may have, such as diabetes
Give up smoking if you are a smoker
Get support for your mental health if you need it
Tell your doctor if symptoms are bothering you, as there are many ways to relieve them

Living well