• Bile duct cancers are treated by members of the BC Cancer Agency’s Gastrointestinal (GI) Tumour Group.
• Other names/types of bile duct cancer: cancer of the bile duct, cholangiocarcinoma, intrahepatic bile duct cancer, distal bile duct cancer, extrahepatic bile duct cancer, perihilar bile duct cancer, Klatskin tumour.
• The bile duct is a 4-5 inch long tube that carries bile from the liver to the small intestine. Bile is a fluid made in the liver to help digest fats in the small intestine.

What causes it and who gets it?
Listed below are some of the known risk factors for this cancer. Not all of the risk factors below may cause this cancer, but they may be contributing factors.

• Bile duct cancer is relatively rare.
• People older than 65 years are more likely to develop bile duct cancer.
• People with chronic inflammation of the bile duct, called sclerosing cholangitis, have an increased risk of bile duct cancer.
• Some diseases, such as polycystic liver disease, ulcerative colitis, stones in the bile duct, choledochal cysts, cirrhosis (damage to the liver) and Caroli syndrome, may increase the risk of bile duct cancer.
• Being overweight is a risk factor.
• There is a much higher rate of bile duct cancer in South East Asia.  This is probably because of the prevalence of infection by liver flukes, which are parasites that live in the bile duct.
• Infection with hepatitis B or hepatitis C may increase the risk of bile duct cancer.
• Exposure to hazardous materials, such as dioxins, asbestos, nitrosamines and Thorotrast, is a risk factor.
• Bile duct cancer is more common in some families with a history of the disease.
• Statistics:
• BC: 115 new cases and 99 deaths in BC in 2005. 
• Canada: Canadian statistics are not available at this time.

Can I help to prevent it?

• Avoid excessive use of alcohol because it may lead to cirrhosis (damage to the liver).
• Hepatitis B may lead to cirrhosis, so vaccinations are encouraged to protect against hepatitis B.
• Avoid exposure to hazardous chemicals.
• If travelling in parts of the world where liver flukes are common, drink only purified water and foods that have been thoroughly cooked.

Screening for this cancer 

• No valid screening test or process is currently available for bile duct cancer.

Signs and Symptoms

• Jaundice (yellowing of the skin and whites of the eyes).
• Itching of the skin.
• Light or clay-coloured stools.
• Unexplained weight loss.
• Loss of appetite.
• Fever.
• Abdominal pain.
• Bleeding.

Diagnosis
Below is a list of some or all of the tests used to diagnose this type of cancer. We have more information on other diagnostic tests for cancer. 

• Physical examination.
• Ultrasound.
• CT scan.
• ERCP - Endoscopic Retrograde Cholangiopancreatography - A tube is passed down the throat and into the bile duct. A dye is injected into the tube, which can show a narrowing or blockage of the bile duct. A brush can also be inserted into the tube to collect cells or tissue for a biopsy.
• Cholangiography – A dye is injected into the bile duct with a thin needle and x-ray pictures are taken. This test can show where the tumour is located.
• Angiography – A small tube is inserted into a blood vessel, dye is injected and a series of x-ray images is then taken. Angiography is used to show the location of blood vessels near the tumour.
• MRI
• Surgery or biopsy may be needed to confirm diagnosis.

Types & Stages
Staging describes the extent of a cancer. The TNM classification system is used as the standard around the world. In general a lower number in each category means a better prognosis. The stage of the cancer is used to plan the treatment.

• T describes the site and size of the main tumour (primary);
• N describes involvement of lymph nodes;
• M relates to whether the cancer has spread (presence or absence of distant metastases).

Stages

Stage 0	The cancer is limited to the innermost layer of the bile duct.
Stage IA	The cancer is limited to the bile duct.
Stage IB	The cancer has spread through the wall of the bile duct.
Stage IIA	The cancer has spread to the liver, gallbladder, pancreas, or the right of left branches of the hepatic artery or to the right or left branches of the portal vein.
Stage IIB	The cancer has spread to lymph nodes, but not to organs far from the bile duct.
Stage III	The cancer has spread to the portal vein, or the hepatic artery, or duodenum (part of the small intestine), gallbladder, colon, or stomach and may have spread to lymph nodes, but has not spread to organs far from the bile duct.
Stage IV	The cancer has spread to organs far from the bile duct.

Types

• Adenocarcinoma
• Over 90% of bile duct cancers are adenocarcinomas.
• Adenocarcinoma begins in the mucus glands lining the inside of the bile duct.
• Bile duct adenocarcinoma is also called cholangiocarcinoma.
• Squamous cell carcinoma.
• Sarcoma.
• Bile duct cancers are generally divided into 3 groups based on their location:
• Intrahepatic – in the bile duct branches inside the liver.
• Extrahepatic or distal – in the common bile duct outside the liver near the small intestine.
• Perihilar or Klatskin tumour – in the hepatic duct where the bile ducts join just outside the liver.

Treatment
Cancer therapies can be highly individualized – your treatment may differ from what is described below.

Surgery

• The preferred treatment is surgery to remove the tumour.  This is possible in 30% - 40% of patients.
• If the tumour can be removed, patients have a 20% - 30% chance of a normal lifespan.
• If the tumour cannot be removed, surgery may be performed to bypass a blockage or a stent (plastic or metal tube) may be inserted into the bile duct to keep it open.

Radiation Therapy

• Radiation therapy may be used if the tumour is fairly small and cannot be removed by surgery.
• It can also be used sometimes to help with symptoms such as pain.

Chemotherapy

• The role of chemotherapy is still being studied.
• Chemotherapy is sometimes helpful, although it will not cure bile duct cancer.

Advanced Bile Duct Cancer

• Many patients with apparently advanced disease can enjoy a prolonged period of palliation by using a combination of surgery and radiation therapy.

Followup after Treatment

• Guidelines for followup after treatment has ended have been developed by the BC Cancer Agency and are listed on our website.
• You will be returned to the care of your family doctor for regular followup.
• Followup testing is based on your type of cancer and your individual circumstances.

Coping with Cancer

• BC Cancer Agency staff can help with quality of life issues for those living with or affected by cancer. This includes the physical, emotional, psychological and practical aspects of care. Each cancer experience is different, but in one way or another, many cancer patients share the same needs.
• The effects of cancer and its treatment can present unique challenges: from practical concerns like money and housing, to emotional concerns like anxiety and grief. If you need support with the practical and emotional impacts of cancer, or in managing symptoms and side effects you can use the information in Coping with Cancer to connect to these resources.

Recommended websites
The following websites have been selected and evaluated by the BC Cancer Agency.

1. NCI http://www.cancer.gov/
2. CCS http://www.cancer.ca/
3. ASCO http://cancernet.org/
4. BC Cancer Agency Recommended Links http://www.bccancer.bc.ca/PPI/RecommendedLinks/typesofcancer/bileduct.htm

Can I help with research at BCCA?
BCCA cancer patients are very helpful when it comes to the fight against cancer. Here are a few ways that you can help.

• Clinical Trials – join our treatment-based research. Ask your oncologist if you qualify for a clinical trial.
• What are the pros and cons of joining a clinical trial?
• Tumour Tissue Bank – support our research by donating some of your cancer tissue.
• BC Cancer Foundation – support our research with a donation of money.