Biliary (Bile Duct) Cancer

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Also known as cholangiocarcinoma, biliary cancer is found in the bile ducts. The bile ducts are a series of branching tubes that carry bile, a digestive fluid, from the liver to the gallbladder and on to the small intestine. Bile duct cancer is classified by its location.

  • Intrahepatic bile duct cancer develops in the bile duct branches located in the liver.
  • Perihilar (hilar) bile duct cancer develops where the branches of the bile duct join and leave the liver. This is the most common type.
  • Distal bile duct cancer develops farther down the bile duct in the area near the small intestine.


In the early stages, there may not be any symptoms. As the cancer develops, symptoms may include:

  • Jaundice (yellowing of the skin and whites of the eyes)
  • Abdominal pain
  • Fever
  • Weakness, fatigue
  • Loss of appetite
  • Unintentional weight loss
  • Itchy skin
  • Dark urine or pale stool


The exact cause is unknown, though gene abnormalities may lead to the growth of cancer cells. Many different gene defects, such as p53 and K-ras abnormalities, have been identified in tumors.  

Risk Factors

Some factors that may increase the risk of bile duct cancer include:

  • A chronic condition that causes inflammation of the bile duct including primary sclerosing cholangitis, ulcerative colitis, bile duct stones and choledochal cysts
  • Cirrhosis of the liver or other condition affecting the liver or bile duct, including polycystic liver disease and Caroli syndrome
  • Infection by parasites called liver flukes (more prevalent in Asia)
  • Being 65 or older 
  • Being overweight or obese
  • Exposure to a radioactive substance or toxic chemical
  • Family history of bile duct cancer
  • Smoking
  • Diabetes
  • Hepatitis B or C
  • Pancreatitis (inflammation of pancreas)


You may be able to lower your risk of bile duct cancer by:

  • Achieving and maintaining a healthy weight
  • Eating a nutritious diet 
  • Exercising regularly
  • Getting a hepatitis B vaccine and avoiding/limiting alcohol (in attempt to prevent cirrhosis)
  • Not smoking
  • Avoiding exposure to toxins


Your doctor will give you a physical exam, and ask about your symptoms and medical history. Blood tests can help evaluate how the liver and gallbladder are functioning, as well as identify any indicators of cancer.Diagnostic tests and procedures may include: 

  • Ultrasound
  • CT scan
  • MRI scan
  • PET scan
  • Endoscopic retrograde cholangiopancreatography (ERCP)
  • X-rays and a thin, flexible tube with a camera (an endoscope) are used to examine the bile duct.
  • Angiography (X-rays are taken of the blood vessels).
  • Laparoscopy (A thin tube with a camera on the end examines the bile duct).
  • Biopsy (A sample of bile duct cells is removed and examined).


Your treatment plan is based on your overall health as well as the size, stage and location of the tumor. Your doctor may recommend a combination of the following surgical and non-surgical treatments. 


Surgery may be performed to try to remove the cancer. Because the bile duct extends into many organs, surgery may involve more than one organ. 

  • For intrahepatic bile duct cancer, surgery may require the removal of part of the liver or an entire lobe.
  • For perihilar bile duct cancer, surgery may involve the removal of part of the liver, bile duct, and gallbladder.
  • For distal bile duct cancer, surgery may involve the removal of part of the pancreas and small intestine.

If the cancer cannot be surgically removed, other types of procedures may help alleviate symptoms. For example, a small tube may be placed inside a cancerous bile duct to facilitate the flow of bile.

Radiation Therapy

Radiation therapy is used to destroy cancer cells and shrink tumors. There are several different types of radiation therapy.

  • External radiation therapy is directed at the tumor from an outside source. 
  • Internal radiation therapy uses radioactive materials placed into the body in or around the cancer cells.
  • Radiation may be done along with surgery. It may also be the main treatment if the tumor cannot be removed.


Chemotherapy is the science of using drugs to destroy cancer cells. This treatment may be used before or after surgery. If the tumor cannot be removed, chemotherapy may be administered alone or in combination with radiation therapy.

Clinical Trials

You may want to consider a clinical trial if treatment options are limited. Ask your doctor if you may be eligible for any clinical trials.