What is bile duct cancer?
The bile ducts, together with the gallbladder, form the biliary system. This is a network of ducts within the liver, and includes the common bile duct outside the liver.
Within the liver there are smaller ducts, similar in size and appearance to capillaries, whose job it is to drain bile into the larger ducts. These small ducts are the intrahepatic ducts, and they are on the left and right of the liver. They join together just outside the liver to form the common bile duct (extrahepatic bile duct). This carries bile from the liver to the gallbladder and down to the small intestine.
Bile, carried by the bile ducts, is a yellowish-green fluid which is made in the liver and stored in the gallbladder. Bile is released whenever we eat fat so that we can digest it more easily. Bile also helps get rid of a substance called bilirubin, which is a waste product formed when red blood cells break down. Bilirubin is what gives bile its colour.
The gallbladder acts as a reservoir that stores bile until food reaches the small intestine. It is then released to help break down the food. The gallbladder is attached to the liver by a small duct, called the cystic duct. Bile duct cancer can occur in the small bile ducts within the liver, the big ducts in the centre of the liver (the hilum) or in the common bile duct draining out of the liver.
Risk factors, signs and symptoms
What are the risk factors for bile duct cancer?
Bile duct cancer is rare. The most common risk factors, particularly those that cause long term inflammation or irritation within the biliary system, are:
You can get bile duct cancer at any age but most people who get it are over 65.
People with PSC have inflammation of the bile ducts, which increases the risk of bile duct cancer (between 8 and 30% of adults with PSC go on to develop bile duct cancer).
These are rare and usually present at birth. They grow slowly, fill with bile and can become cancerous.
If these remain in the biliary system for a long time, they can cause irritation to the duct lining and increase the risk of bile duct cancer.
This includes Thorotrast, which was an X-ray contrast agent used several decades ago.
This is more common in South East Asia, and is caused by eating raw river fish infected with flukes. Once eaten, these flukes accumulate in the bile ducts, where they can live for many years, causing infection and more rarely, cancer. There is only a very small risk of liver fluke in the West, where most bile duct cancers are ‘sporadic’ – that is, they occur with no known or obvious cause.
Other possible risk factors for bile duct cancer:
There is some evidence that long term liver damage of any cause, such as chronic viral hepatitis B or C, and alcohol misuse, may also be risk factors. Obesity, diabetes, fatty liver disease and smoking are also thought to be linked to bile duct cancer.
What are the signs and symptoms of bile duct cancer?
Bile duct cancer causes few symptoms in its early stages, and those that do occur tend to be quite vague, such as nausea and loss of appetite. If the cancer in the bile ducts disrupts the flow of bile, it flows back into the bloodstream and other tissues of the body, causing symptoms such as:
- Jaundice – yellowing of the skin and whites of the eyes. This is because of the excess of bilirubin in the body, which is what causes the yellow tinge. Jaundice is the most common symptom, but can develop either early or late, depending on where the cancer is.
- Itchy skin. This is caused by a build-up of bile salts.
- Dark urine. Urine (wee) is usually the colour of straw, or yellow. Dark urine indicates a build-up of waste products in the body, such as bile.
- Pale stools. Bile salts are what make your stools (poo) brown, so if it’s pale or clay-coloured, it may mean the flow of bile is blocked.
- Unexplained weight loss.
- Abdominal pain and swelling – the pain may radiate towards your back as the cancer progresses.
- High temperature (fever).
You may also experience loss of appetite and feel more tired than normal (fatigue). These symptoms can be caused by lots of other things, but it’s important to see your doctor and get them checked out.
How is bile duct cancer diagnosed?
It’s likely you’ll need various tests to help diagnose bile duct cancer. Usually, you’ll see your GP first, who will examine you and may take blood tests. These tests might include:
- A full blood count (FBC). Blood is made up of red blood cells, white blood cells and platelets. A full blood count looks for anything unusual in any of these that may need further investigation.
- A liver blood test, which may also be referred to as a liver function test. Liver experts feel that the term ‘liver blood test’ is more reflective of what the tests actually do – that is, look for ‘markers’ in the blood that indicate a problem with the liver, rather than assessing how well the liver is working. This test is used to measure the levels of proteins, liver enzymes and bilirubin in the blood. Any abnormalities in any of these may indicate a problem with the liver and will require further investigation.
Your GP will refer you to a hospital specialist, who will ask about your medical history and arrange for you to have the blood tests outlined above if they haven’t been done by your doctor. The following tests may also be used to diagnose bile duct cancer:
This is a quick and painless examination that uses soundwaves to make up pictures of the bile ducts and surrounding organs. You will usually be asked not to eat or drink anything for six hours before the scan and will need to lie down comfortably on your back while a gel is spread over your tummy. The gel makes it easier to use the ultrasound probe (also known as a transmitter or receiver unit) as it moves over the skin, and improves the quality of the images it sends back to the computer.
A CT scan takes a series of x-rays, which builds up three-dimensional images of the inside of the body. You’ll be asked not to eat or drink anything for four hours before your scan, and you may be given a drink or injection of medical dye to highlight the particular areas of the body the doctors need to see clearly. CT scans are completely painless and take about 30 minutes, after which you can usually go home.
An MRI scan uses magnets and radio waves to produce images of the inside of the body. It creates clear pictures of the soft tissues inside the abdomen (tummy), showing them from all angles (cross-sectional). This allows doctors to check for any abnormalities they wouldn’t be able to find from a physical examination, or without having to operate.
You may have a special type of MRI scan, called an MRCP (magnetic resonance cholangiopancreatography). This helps doctors clearly see the bile ducts. You’ll be asked to remove any metal jewellery or other types of clothing (such as on a belt buckle) and may then be given an injection of what’s called a contrast medium into a vein in your arm. This is a dye that helps images from the scan show up more clearly. The MRI scanner itself is a long tube, and you’ll be asked to lie down inside it for about 30 minutes. An MRI scan isn’t painful but it is noisy, although you’ll be given earplugs or headphones so you can listen to music. You’ll be able to hear and speak to the person operating the scanner at all times.
This is where a doctor uses a long, thin tube called an endoscope to look at your bile ducts. You’ll be asked not to eat or drink anything for about six hours before you have the test, so that your stomach and the first part of your small intestine (bowel) are empty. Then you’ll be given a sedative, usually via a cannula (thin tube that’s inserted into the vein) to relax you (this can be topped up as needed during the procedure). You will have a local anaesthetic spray at the back of your throat to numb the area.
The doctor will pass the endoscope down your throat, into your stomach and the small bowel so they can look carefully at the bile ducts. They will inject a dye into the bile ducts that helps to show up any blockages or abnormalities. If a blockage is found, the doctor may insert a small tube called a stent, made of either plastic or metal, into the bile duct to help bile drain away.
You’ll be given antibiotics before having an ERCP to help prevent infection, and may be required to stay in hospital overnight.
This is similar to the scan above, except that a doctor will attach a very small ultrasound probe to the top of the endoscope, which takes an ultrasound scan of the gallbladder and bile ducts through the wall of the small bowel.
This procedure helps show up any blockages or abnormalities inside the bile duct. It involves a local anaesthetic injection in your tummy to numb the area over your liver, after which the doctor will insert a very fine needle through the skin. Dye is injected into a bile duct inside the liver, so that it shows up more clearly on an X-ray. It is also possible to get sample of tissue (biopsy) from the liver using this procedure, and doctors can drain bile away using this technique by inserting a stent. You’ll be given antibiotics before and after to help prevent infection, and have to stay in hospital for at least one night.
This is where a small sample of tissue is removed from the bile duct and examined for signs of cancer. It can be done via PTC or ERCP (see above). Your doctor may use an ultrasound or CT scan at the same time to make sure they take tissue samples from the right place
This is a where a surgeon examines your bile ducts using a tube with a camera attached (a laparoscope) to look inside the bile ducts and at the surrounding areas. It’s carried out under general anaesthetic, which means you won’t be able to eat or drink anything for six hours before the procedure. The surgeon will make a small cut (incision) in your tummy and insert the camera through it to see your bile ducts clearly. Afterwards you’ll have stitches and a dressing on the wound. You may be able to go home the same day, although sometimes a laparoscopy requires an overnight stay in hospital.
This is a term doctors use to explain the size and location of the cancer so they can work out the best treatment plan for you. There are different ways of staging cancer, but the TNM staging system is the one normally used in staging bile duct cancer.
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 any other part of the body. This is known as metastatic, or secondary, cancer.
Grading a cancer is when doctors look at the cells under a microscope to see how quickly the cancer might develop. In low-grade tumours, the cells look very much like normal cells, are slow-growing and much less likely to spread. In high-grade tumours, the cells look very abnormal. They are likely to be fast-growing, and to spread.
How is bile duct cancer treated?
The treatment your doctor recommends for bile duct cancer will depend on several things, such as the size and position of the cancer, and whether it has spread. They will also take into account your general health.
Surgery offers the only potential complete cure for bile duct cancer. But it may not be an option for many patients, as the disease may be too advanced or the patient may be considered too poorly for such a major operation.
Surgery can be used to try to remove all of the cancer or to relieve symptoms. The location of the tumour can affect the surgical treatment, so it is very important that the patient is properly evaluated by an experienced surgeon who specialises in biliary cancer surgery to decide on the correct option.
If surgery is possible, the type of operation that is done depends on the size of the cancer and whether it has begun to spread into nearby tissues:
- Removal of the bile ducts - If the cancer is at a very early stage (stage 1), just the bile ducts containing the cancer are removed. The remaining ducts in the liver are then joined to the small bowel, allowing the bile to flow again.
- Partial liver resection - If the cancer has begun to spread into the liver, the affected part of the liver is removed, along with the bile ducts.
- Whipple procedure - If the cancer is larger and has spread into nearby organs, the bile ducts, part of the stomach, part of the small bowel (duodenum), the pancreas, gall bladder and the surrounding lymph nodes may need to be removed.
Depending on the type of surgery you have, you’ll stay on an intensive care ward for the first night or two before being moved to a general ward to recover. Most people are in hospital for a week or two after this kind of operation.
A stent (usually made of metal ) is inserted into the bile duct to hold it open and let bile flow from the liver to the intestines. A stent is a tube about as thick as a ball-point pen refill and 5-10cm long (2-4 inches). A stent may be put in using an ERCP or occasionally a PTC procedure.
This will relieve the symptoms of jaundice, your general health will improve and you will able to digest food again normally. Stents usually need to be replaced every three to four months as they can become blocked, and cause a biliary tract infection (cholangitis), which can rapidly become serious. Symptoms of a blocked stent/infection include:
- high temperature/fever
- chills, shivering.
If these symptoms develop, it is important to contact your doctor or CNS (Clinical Nurse Specialist) for advice, as antibiotic treatment may be needed and the stent may need to be replaced.
This is when doctors use anti-cancer drugs (known as ‘cytotoxic’) to destroy cancer cells. The chemotherapy treatments for those with inoperable bile duct cancer include:
- a combination of Gemcitabine (Gemzar®) and Cisplatin
- modified folinic acid, 5-fluorouracil and oxaliplatin (mFOLFOX)
These treatments won’t cure the cancer but aim to to shrink or slow the growth of the cancer, and to relieve symptoms. If surgery has been possible, then a course of chemotherapy (currently capecitabine) may be recommended to help prevent the cancer coming back.
Targeted treatments specifically attack the things that make cancer cells different to your healthy cells and that help them to survive.
Pemigatinib has been approved for bile duct cancer that continues to get worse after having chemotherapy.
Pemigatinib works by targeting a certain genetic signature found in about 1 in 10 cases of bile duct cancer. This is called a fibroblast growth factor receptor 2 (FGFR2) fusion or rearrangement, which means that the DNA instructions in the cancer cells have got joined together or moved around in a way that tells the cancer cell to keep making more copies of itself.
Pemigatinib will only work against cancers that have this FGFR2 signature. To help decide if this treatment is suitable for you, your doctor will take a sample of your cancer and test it for the FGFR2 signature. This is called molecular diagnosis.
Pemigatinib doesn't aim to cure bile duct cancer. Instead it aims to keep it under control, reduce symptoms, and extend your life.
Radiotherapy is not often used to treat bile duct cancer, although it may be combined with other treatments to help make them more effective. Radiotherapy is usually given externally, via a machine that uses high-energy rays to destroy cancer cells.
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