Bile Duct Cancer (Cholangiocarcinoma)
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Identify the bile duct system
The bile ducts are part of the digestive system. They are the tubes that connect the liver and gall bladder to the small bowel. Bile ducts carry bile, which breaks down fats in food to help us digest them. Bile is made by the liver and a small portion is stored in the gall bladder. The bile ducts and gall bladder together are known as the biliary system. The bile duct system is divided to various parts for descriptive purposes.
- Intra-hepatic – the bile ducts inside the liver
- Extra-hepatic – the bile ducts outside the liver.
The extra-hepatic bile ducts are divided into two parts:
- Hilar – where the left and right hepatic ducts meet, just below the liver
- Distal – the lower part of the bile ducts, nearest to the bowel.
What is Bile duct cancer (cholangiocarcinoma)?
Bile duct cancer (cholangiocarcinoma) is a type of cancer that starts in the lining of the bile duct system.
What causes bile duct cancer?
The causes of most bile duct cancers are unknown. But there are some factors that can increase your risk of developing it.
- Age - Most cases are diagnosed over the age of 60
- Inflammatory conditions - Long-term inflammatory conditions can increase the risk of bile duct cancer. For example: ulcerative colitis, primary sclerosing cholangitis
- Abnormal bile ducts - People born with bile duct abnormalities, such as choledochal cysts, have a higher risk of developing bile duct cancer.
- Chronic liver disease - People who have cirrhosis have an increased risk of developing bile duct cancer
- Infection - Infection with a parasite called the liver fluke can cause bile duct cancer
What are the symptoms of bile duct cancer?
Cancer in the bile ducts can block the flow of bile from the liver to the bowel. This means that the bile flows back into the blood and body tissues. This cases the main sign and symptoms which can include:
- the skin and whites of the eyes turning yellow (jaundice)
- urine turning dark yellow
- stools (bowel motions) looking pale
- itchy skin
Other possible symptoms include:
- discomfort in the tummy area (abdomen)
- loss of appetite
- feeling generally unwell
- high temperatures (fevers)
- weight loss
These symptoms can be caused by many things other than bile duct cancer. But it is important to get them checked by your doctor.
How bile duct cancer is diagnosed?
You usually begin by seeing gastroenterological specialist doctor. The doctor will ask you about your symptoms and any previous medical problems. They will also examine you and take blood samples to check your general health and that your liver is working properly. The doctor might use the following tests to diagnose bile duct cancer.
This uses soundwaves to make up a picture of the bile ducts and surrounding organs. You will usually be asked not to eat or drink anything for at least six hours before the scan. The test is painless and only takes a few minutes.
MRI (magnetic resonance imaging) scan
This test uses magnetism to build up a detailed picture of areas of your body. Doctors may use a special type of MRI scan called an MRCP (magnetic resonance cholangiopancreatography). This scan helps the doctors to see the bile ducts.
CT (computerised tomography) scan
A CT scan takes a series of x-rays, which build up a three-dimensional picture of the inside of the body. The scan takes 10 to 30 minutes and is painless.
ERCP (endoscopic retrograde cholangio-pancreatography)
This is an endoscopic procedure done under anaesthesia and it is used to take an x-ray picture of the bile duct while being used to unblock the bile duct.
EUS (endoscopic ultrasound scan)
This scan is similar to an ERCP, but the doctor attaches an ultrasound probe to the endoscope. This takes an ultrasound scan of the gall bladder and surrounding areas.
Your doctor will usually recommend having a biopsy of the tumour especially if you are being considered for chemo-radiotherapy before operation or if your treatment is mainly chemo-radiation. This is to confirm the diagnosis. It involves taking some cells or tissue samples from the affected area of the bile duct. Then they look at the biopsy sample under a microscope. They may take a biopsy during an ERCP or PTC. You may have a CT or ultrasound scan at the same time, to make sure the biopsy is taken from the right place.
A procedure called a laparoscopy is sometimes used to help diagnose bile duct cancer. It is carried out under a general anaesthetic, so you are not awake. The surgeon uses a tube with a tiny camera attached, called a laparoscope, to look inside your tummy (abdomen). They make a small cut in your tummy. They then put the laparoscope into the tummy through this cut. This lets them examine the bile duct and the tissue around it for cancer.
The stage of a cancer describes its size and whether it has spread beyond its original site. Knowing the stage of the cancer helps the doctors plan the best treatment for you.
Cancer can spread through the bloodstream or the lymphatic system. The lymphatic system is part of the body’s defence against infection and disease. It is made up of a network of lymph nodes connected by thin tubes. Lymph nodes are small bean-shaped structures. Your doctors will usually examine the lymph nodes close to the bile ducts. This helps them to find out the stage of the cancer.
The TMN staging system is the one usually used for bile duct cancer.
TNM staging system
- T describes the size of the tumour
- N describes whether the cancer has spread to the lymph nodes
- M describes whether the cancer has spread to another part of the body (known as metastatic or secondary cancer).
This system gives detailed information about the tumour stage. The staging of the cancer is different depending on the part of the bile duct where the cancer started. Your doctor will be able to tell you more about your situation.
Grading gives an idea of how quickly a cancer may develop. It is based on the way cancer cells look under a microscope:
- In low-grade tumours, the cancer cells look very much like normal cells. They are usually slow-growing and are less likely to spread.
- In high-grade tumours, the cells look very abnormal. They are likely to grow more quickly and are more likely to spread.
What is the treatment for bile duct cancer?
The treatment you have will depend on:
- the position of the cancer
- the size of the cancer
- whether it has spread beyond the bile duct
- your general health
In some situations, the aim of treatment is to relieve symptoms.
Surgery for bile duct cancer
One of the main treatments for bile duct cancer is surgery. Surgery may be done to:
- try to remove all of the cancer
- relieve symptoms.
The type of surgery you are offered will depend on:
- the size of the cancer
- whether it has begun to spread into nearby tissues
- your fitness and general health.
Surgery to remove all the cancer
Surgery may be able to cure the cancer for some people. But this is a major operation and is not always suitable. The surgery is complex and is carried out by a surgeon who is a specialist in bile duct cancer. Your surgeon will look at the stage of the cancer to see if this type of operation is possible:
- If the cancer is at a very early stage – the surgeon will remove only the bile ducts containing the cancer. They then join the remaining ducts in the liver to the small bowel. This allows the bile to flow again.
- If the cancer is in the bile ducts in the liver (intrahepatic bile duct cancer) – the surgeon will remove the affected part of the liver and the bile ducts.
- If the cancer is where the left and right hepatic ducts meet just below the liver (perihilar bile duct cancer) – the surgeon will remove the gall bladder and the part of the liver close to the bile ducts. Sometimes the surgeon will also remove part of the pancreas and part of the small bowel.
- If the cancer is in bile ducts near the pancreas and the small bowel (distal bile duct cancer) – the surgeon will remove the bile ducts, gall bladder, part of the pancreas and part of the small bowel (duodenum). This is sometimes called a Whipple’s operation.
After your operation you may need to stay in an intensive care ward for the first couple of days. You will then move to a general ward until you recover. Most people need to be in hospital for about 1 to 2 weeks after these types of operation.
Further treatment after surgery to remove the cancer
If it was not possible to remove all the cancer during the operation, you may be offered extra treatment. The aim is to reduce the risk of the cancer coming back. This is called adjuvant treatment. It may involve chemotherapy, radiotherapy or a combination of both, called chemoradiation. Doctors are not sure if adjuvant treatment is effective and these treatments can cause side effects. If your doctor offers you adjuvant treatment, they will talk to you about the possible benefits and disadvantages. This can help you decide whether to go ahead.
Surgery to relieve blockage (obstruction)
If the part of the small bowel called the duodenum is blocked, it can cause sickness (vomiting). This may be helped with an operation to bypass the blockage. The surgeon does this by connecting the stomach to the next section of small bowel (the jejunum).
Sometimes bypass surgery may be done to relieve jaundice caused by a blocked bile duct. But it is more common to have stent insertion. This does not need an operation.
A stent is a tube that your doctors can put into the bile duct to hold it open. This allows bile to drain away. The tube is thin, and about 5 to 10cm (2 to 4 inches) long. The doctors may put the stent in using an ERCP procedure, or sometimes a PTC procedure:
The ERCP method – the preparation and procedure is the same as for ERCP. By looking at x-rays, the doctor can see the narrowing in your bile duct. They stretch the narrowed area using dilators (small, inflatable balloons). They then put the stent in through the endoscope, so the bile can drain. If you have any discomfort while this is being done, it is important to let your doctor know.
The PTC method – the procedure and preparation is the same as for PTC. Your doctor passes a wire into the blocked area and then guides the stent along the wire. Sometimes they will put a drainage tube (catheter) in the bile duct. One end of the tube is in the bile duct. The other end stays outside the body, connected to a bag that collects the bile. This can help the doctor insert the stent. They usually leave the tube in for a few days. Once they remove it, the area heals in 2 to 3 days.
Sometimes a combination of ERCP and PTC may be used.
Chemotherapy for bile duct cancer
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It may be given if you cannot have surgery or if cancer comes back after surgery. The aim is to try to shrink or slow the growth of the cancer and to relieve symptoms. Sometimes chemotherapy may be used after surgery.
Doctors often use a combination of the drugs cisplatin and gemcitabine (Gemzar®) to treat bile duct cancer.
Radiotherapy for bile duct cancer
Radiotherapy treats cancer by using high-energy rays to destroy cancer cells, while doing as little harm as possible to normal cells. It is usually given externally from a machine outside the body. But it can also be given internally by placing radioactive sources inside the body.
Radiotherapy is not often used to treat bile duct cancer. But sometimes it is given on its own or in combination with chemotherapy to:
- try to lower the risk of cancer coming back after surgery
- help relieve symptoms.
- Doctors are still trying to find out how effective radiotherapy or chemoradiation is in treating bile duct cancer. Your doctor will talk to you about possible benefits and disadvantages if you are offered this treatment.
Selective internal radiotherapy treatment (SIRT)
SIRT is a way of giving internal radiotherapy. It may be used to help relieve symptoms of cancer.
The treatment involves having tiny beads (microspheres) injected into the liver. Each bead is coated with a radioactive substance that gives off radiation. The treatment gives a dose of radiotherapy to the liver over a few days.
The microspheres are harmless and they stay in the liver permanently. The treatment involves staying in hospital for 1 to 4 days.
Side effects include having a high temperature and abdominal pain straight after the injection. These can last for a few days. Other side effects include feeling sick (nausea), being sick (vomiting), and diarrhoea.