Cholangiocarcinoma (bile duct cancer)
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
Transcript
I think back to that time, that day of diagnosis and those precious weeks over Christmas and New Year and our daughter was in England and we wouldn’t tell anyone until Clare had come back. So you’re really sort of living in this, this really tender space that is marked by occasion because it’s Christmas and trying to not be sad that this is probably my last party.
I live in Sydney, I live in the Inner West. My husband and I met at Carols by Candlelight by chance a long time ago and we’ve lived in England and then we came back because there was a wonderful oncology nurse job that I was offered and applied for.
Yeah I’m really lucky, I’ve got a wonderful family, three adult children who are the best thing since sliced bread.
Like everybody I was really busy at work but I’d noticed that I could get a bit breathless and I developed a cough.
I stopped walking Fergus because I was just too tired. So due to me being unwell we’d already had to shave two weeks off our trip to Europe and thank God we were able to go to Venice, really looking forward to being there, enjoying being on the water but also the full moon was there when we were in Venice and really keen to get some really good photographs from the bridges. It was so disappointing just not being able to manage the bridges to get those really good photographs.
My diagnosis was a long time coming, so in the March I saw my GP and was referred to a respiratory physician, had a full respiratory workup, there were no issues or complaints there, nothing explained why I had this cough. I saw a cardiologist and had the full monty of investigations and then right at the end had a heart MRI and that showed something on my liver.
I’d had the liver scan on the Friday, I got a call saying the doctor wanted to see me that afternoon.
The GP gave the size of the lesion and it was the size of your fist, so that great big thing had been pushing up and growing in my liver to distort, so that’s why I had this cough and that’s why I was so short of breath.
I was seeing the surgeon the next day. The tumour involves two major veins so surgery was out of the question. I needed to have chemotherapy to shrink it down and I started chemotherapy at the end of January. So the plan was to have four cycles of chemotherapy and immunotherapy and scan, and then hopefully have surgery. And the tumour didn’t budge at all with treatment, so the best way to go forward was just to continue with the chemotherapy and immunotherapy.
Fortunately, during that time I had had access to the MOST trial and that’s where the gene mutation was identified. How it played out was that I finished the chemotherapy and immunotherapy and went straight to trial because the chemotherapy just did not change the size of the tumour.
So it was fantastic being on this trial and within two months the tumour had shrunk by a bit more than a third, so that was the first time that there was something to get actually excited about and think gosh, you know, we could plan things, we could anticipate things and but it comes at a price because there are side effects. I was on it for a year and it broke through well you know there were new secondaries and I was so lucky that my oncologist had access to another drug that was similar to the trial drug but no one in Australia had been on it yet and I’m still on that drug now.
What you have to live with. I didn’t have a nurse. How do you navigate all the things that you have to navigate now? I had this army of wonderful friends but they’re my friends and they are just stressed that their dear friend has a Cholangio and I can’t stress how happy I am to know the Liver Foundation has a specialist nurse. That is gold and hopefully nurse will grow to nurses and what that will bring to patients.
I think it’s wonderful that the Liver Foundation is providing this type of care. I have the most wonderful life and I look forward to lots of things, I have lots of joy, lots of happiness, wonderful family and friends and it’s great to be able to tell my story.
And I did go back to Venice and there’s a great photo of me and that absolute feeling of pure joy that I did this, I’ve mastered that and it’s because of being on the clinical trial.
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
Additional information and support
Cholangiocarcinoma Foundation Australia
References
Cholangiocarcinoma Foundation Australia. About Cholangiocarcinoma <https://cholangiocarcinomaaustralia.org/about-cholangiocarcinoma/>
Australasian Gastro-Intestinal Trials Group (AGITG.) What is cholangiocarcinoma? <https://gicancer.org.au/news/what-is-cholangiocarcinoma/>
Mayo Clinic. Cholangiocarcinoma (bile duct cancer) <https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/symptoms-causes/syc-20352408>
Pancare Foundation. Diet and nutrition <https://pancare.org.au/cancer/biliary-cancer/biliary-cancer-diet-nutrition/>
Reviewed June 2024