Gastrointestinal Cancers

Biliary Cancer


Bile duct cancer, also called cholangiocarcinoma, is a rare and slow-growing cancer that forms in the part of the bile duct that is outside the liver. The bile duct is the tube that collects bile from the liver and joins a duct from the gallbladder to form the common bile duct, which carries bile into the small intestine when food is being digested.

Depending on its location, bile duct cancer falls into three groups:

Hilar (perihilar) Bile Duct Cancer
These are the most common types of bile duct cancers. They form in the area where hepatic duct branches leave the liver. In the early stages, only a small portion can be removed by surgery. Hilar bile duct cancers are also referred to as Klatskin tumors.

Intrahepatic Bile Duct Cancer
This form originates within the liver on smaller duct branches. They can easily be confused with other cancers that form within the liver cells. Only 1 out of 10 bile duct cancers are intrahepatic. About half of these cancers can be removed by surgery at the time they are found.

Distal Bile Duct Cancer
More removable than the above mentioned, these bile duct cancers form closer to the small intestine. However, typical surgery for these types of cancers can require part of the pancreas and small intestine to be removed.


When functioning properly, bile ducts drain bile into the small intestines to aid in the digestive process and absorption of fats. When there is an obstruction in this system of tubes, in the case of bile duct cancer, a tumor may block the flow of bile, causing the bile to back up into the liver, causing the fats and lipids to pass through undigested. If this happens, the bilirubin (a brownish-yellow substance found in bile) builds up and causes jaundice, or the yellowing of the skin and eyes. The most common symptoms of bile cancer are characterized by weight loss and changes in stool or urine.

Potential symptoms associated with bile duct cancer include:

  • Yellowing of the skin and whites of the eyes, sometimes accompanied by itchy skin (jaundice)
  • Decreased appetite/weight loss
  • Diarrhea
  • Enlarged abdominal mass/bloated feeling
  • Itching/pruritis
  • Nausea and/or fever
  • Changes in stool or urine (clay/white-colored stool and dark brown urine)

Risk Factors

Potential risk factors associated with bile duct cancer include:

  • Colitis or certain types of liver disease
  • Primary sclerosing cholangitis (with or without a history of Inflammatory Bowel Disease/Ulcerative Colitis)
  • Choledochal cysts
  • Infection with a Chinese liver fluke parasite


Stage 0: The cancer is found only in innermost layer of the bile duct and has not spread to lymph nodes or elsewhere in the body.

Stage I: Still contained within the bile duct, the cancer now extends throughout the layers of tissue. It still has not spread to lymph nodes or beyond the bile duct.

Stage II: The cancer has now spread to nearby organs, such as the liver, gallbladder, or pancreas. It may also be found in the branches of the portal vein or hepatic artery, but has not spread to distant sites. (In Stage IIB, the cancer has also spread to nearby lymph nodes.).

Stage III: The cancer has spread to the main portal vein, the common hepatic artery, duodenum (first part of the small intestine), colon, stomach, or abdominal wall, but not beyond. The cancer may or not have spread to the lymph nodes.

Stage IV: The cancer has spread to distant sites.


Treatment of localized extrahepatic bile duct cancer may include:

  • Stent placement to relieve blockage of the bile duct, which may be done before surgery to relieve jaundice
  • Surgery, with or without external-beam radiation therapy

Treatment of unresectable extrahepatic bile duct cancer may include:

  • Stent placement or biliary bypass with or without internal or external radiation therapy as palliative treatment to relieve symptoms and improve quality of life
  • A clinical trial of hyperthermia therapy, radiosensitizers, chemotherapy, or biologic therapy

Treatment of recurrent extrahepatic bile duct cancer may include:

  • Palliative treatment to relieve symptoms and improve quality of life
  • A clinical trial

The National Cancer Institute provides more detailed information about bile duct cancers and available clinical trials.

National Cancer Institute, Mayo Clinic, Cancer Treatment Centers of America.