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Bile duct cancer

This page was reviewed under our medical and editorial policy by Maurie Markman, MD, President, Medicine & Science

This page was updated on July 20, 2022.

Bile duct cancer is a rare disease, accounting for 10 to 20 percent of all liver cancers. The cancer begins in the bile ducts, which are thin tubes that transport digestive fluid (called bile) from the liver and gallbladder into the small intestine.

About 8,000 people in the United States are diagnosed with bile duct cancer annually, according to the American Cancer Society. When bile duct tumors block the flow of bile and bilirubin from the liver, a person with this cancer may exhibit jaundice, a yellowing of the skin and the whites of the eyes. The disease may also cause symptoms such as itching, abdominal pain and weight loss. Due to the location of the bile ducts deep inside the body, these tumors are rarely caught early. Surgery is typically the first line of treatment.

No bile duct cancer patient is the same. Get personalized treatment.

At City of Hope, our oncologists are trained and experienced in diagnosing and treating bile duct cancers. Our multidisciplinary teams of cancer experts will evaluate the disease to determine its type and stage, using the information to tailor a treatment plan to your needs and diagnosis.

This overview will cover the basic facts about bile duct cancer, including:

If you believe you may be experiencing symptoms of bile duct cancer and want to schedule an appointment for diagnostic testing, or if you’re interested in a second opinion on your bile duct cancer diagnosis and treatment plan, call us or chat online with a member of our team.

What can cause cancer of the bile duct?

Who gets bile duct cancer?

More than 60 percent of bile duct cancer patients are 65 years or older, and most cases do not appear to have a link to family history.

Smoking and obesity may result in an increased risk of bile duct cancer, as may excessive alcohol use. Alcohol use can also cause cirrhosis of the liver and increase a person’s liver cancer risks.


Len A.

Pancreatic Cancer

"My doctor said to me, “You have cancer, and it is bad. But together, we can formulate a plan. I want to help you fight the cancer, and I want you to have hope.” The nurse held my hand, and I knew in that moment that I wanted to get treatment at City of Hope."


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Bile duct cancer types

Cancer can occur in any part of the bile duct. The location of the primary tumor (inside or outside the liver) and the kind of cancer cells involved determine the type of bile duct cancer.

Most bile duct cancers develop in the ducts outside the liver. These are called extrahepatic bile duct cancers and include:

  • Bile duct adenocarcinomas, also called cholangiocarcinoma, which form in the cells of the mucous gland lining the inside of the bile duct
  • Hilar (or perihilar) bile duct cancers, also called Klatskin tumors, which form in the hilum region where hepatic duct branches leave the liver
  • Distal bile duct cancers, which occur near the small intestine

Intrahepatic bile duct cancer begins in the smaller duct branches of the liver. They are sometimes misdiagnosed as liver cancer, though both are typically treated the same way.

Learn more about bile duct cancer types

Bile duct cancer symptoms

Diagnosing bile duct cancer

At City of Hope, we use a variety of tests and tools to diagnose bile duct cancer, evaluating the disease and customizing a treatment plan for each patient. Throughout treatment, imaging and laboratory tests track the size of the tumors, monitor the response to treatment and help facilitate treatment plan modifications when needed.

The following procedures are commonly used for diagnosing bile duct cancer:

Learn more about diagnostic procedures for bile duct cancer

Bile duct cancer treatments

Diagnosis and treatment options at our GI Cancer Centers

We understand that bile duct cancer and other malignancies of the gastrointestinal tract create unique challenges for patients, and that treatment options are very specific to each disease. That’s why each City of Hope hospital has a GI Cancer Center dedicated to diagnosing, treating and supporting the quality of life of patients with bile duct and GI cancers. Committed to offering state-of-the-art treatments for patients with bile duct cancer, our multidisciplinary team of board-certified medical oncologists, surgical oncologists, radiation oncologists and supportive care clinicians work with our patients to deliver quality clinical care with a patient-centered approach. After your diagnosis, your GI Cancer Center care team will discuss your options with you and help you develop a personalized care plan tailored to your individual needs.

Because of the digestive tract’s role in processing food and waste, many patients with gastrointestinal disease have difficulty with digestive function. That’s why nutrition therapy is a key component of our GI Cancer Centers’ approach. Each center is staffed by oncology-trained dietitians who work with patients in developing a healthy, balanced and appetizing nutrition plan. If patients become malnourished, the dietitian is available to help them establish healthy lifestyle and eating habits to help improve their condition.

The GI Cancer Center teams also work closely with other supportive care clinicians to manage additional disease- and treatment-related side effects, such as nausea and vomiting. These supportive care experts may include a pain management physician, naturopathic provider, mind-body provider and spiritual support provider.

Qualified bile duct cancer patients may enroll in carefully selected clinical trials. Your care team will discuss whether you qualify for any of our ongoing clinical trials and, if so, help you enroll.