The following material is adapted from the National Cancer Institute1.
Bile duct cancer is a rare disease in which cancer cells form in the bile ducts. Bile duct cancer is also called cholangiocarcinoma.
A network of tubes, called ducts, connects the liver, gallbladder, and small intestine. This network begins in the liver where many small ducts collect bile (a fluid made by the liver to break down fats during digestion). The small ducts come together to form the right and left hepatic ducts, which lead out of the liver. The two ducts join outside the liver and form the common hepatic duct. The cystic duct connects the gallbladder to the common hepatic duct. Bile from the liver passes through the hepatic ducts, common hepatic duct, and cystic duct and is stored in the gallbladder.
When food is being digested, bile stored in the gallbladder is released and passes through the cystic duct to the common bile duct and into the small intestine.
There are two types of bile duct cancer:
Illustration obtained with permission from © 2022 Terese Winslow LLC. All rights reserved.
Illustration obtained with permission from © 2022 Terese Winslow LLC. All rights reserved.
These and other signs and symptoms may be caused by bile duct cancer or by other conditions. Check with your doctor if you have any of the following:
There are no routine screening tests to check for bile duct cancer before signs and symptoms occur.
Anything that increases your chance of getting a disease is called a risk factor. There are several risk factors associated with bile duct cancer. Not everyone with one or more of these risk factors will develop the disease, and the disease will develop in some people who don’t have any known risk factors. People who think they may be at risk should discuss this with their doctor.
Risk factors for bile duct cancer include the following conditions:
Procedures that make pictures of the bile ducts and the nearby area help diagnose bile duct cancer and show how far the cancer has spread. The process used to find out if cancer cells have spread within and around the bile ducts or to distant parts of the body is called staging.
In order to plan treatment, it is important to know if the bile duct cancer can be removed by surgery. Tests and procedures to detect, diagnose, and stage bile duct cancer are usually done at the same time. Every person will not receive all the tests described below.
The following tests and procedures may be used:
Different procedures may be used to obtain a sample of tissue and diagnose bile duct cancer. Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. The type of procedure used depends on whether the person is well enough to have surgery.
Types of biopsy procedures include the following:
The stage describes the extent of cancer in the body. Knowing the stage helps the doctor plan the best treatment. Bile duct cancer stages are described using a staging system called the TNM staging system.
Intrahepatic bile duct cancer
Illustration obtained with permission from © 2022 Terese Winslow LLC. All rights reserved.
Perihilar bile duct cancer
Distal bile duct cancer
Illustration obtained with permission from © 2022 Terese Winslow LLC. All rights reserved.
This section describes the different types of treatment for bile duct cancer. Which of these treatments a person may receive will depend on whether the cancer can be completely removed with surgery (resectable) or not (unresectable).
Surgery
The following types of surgery are used to treat bile duct cancer:
After the doctor removes all the cancer that can be seen at the time of the surgery, some people may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. It is not yet known whether chemotherapy or radiation therapy given after surgery helps keep the cancer from coming back.
The following types of palliative surgery may be done to relieve symptoms caused by a blocked bile duct and improve quality of life:
Radiation therapy
Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. These are the main ways radiation might be given to treat bile duct cancer:
It is not known whether external radiation therapy helps in the treatment of resectable bile duct cancer. In unresectable, metastatic, or recurrent bile duct cancer, new ways to improve the effect of external radiation therapy on cancer cells are being studied:
External and internal radiation therapy are used to treat bile duct cancer and may also be used as palliative therapy to relieve symptoms and improve quality of life.
Chemotherapy
Chemotherapy uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. There are two main types of chemotherapy used to treat bile duct cancer.
Systemic chemotherapy is used to treat unresectable, metastatic, or recurrent bile duct cancer. The following chemotherapy drugs may be used:
In unresectable, metastatic, or recurrent bile duct cancer, intra-arterial embolization is being studied. It is a procedure in which the blood supply to a tumor is blocked after anticancer drugs are given in blood vessels near the tumor. Sometimes, the anticancer drugs are attached to small beads that are injected into an artery that feeds the tumor. The beads block blood flow to the tumor as they release the drug. This allows a higher amount of drug to reach the tumor for a longer period of time, which may kill more cancer cells.
It is not known whether systemic chemotherapy helps in the treatment of resectable bile duct cancer.
In a liver transplant, the entire liver is removed and replaced with a healthy donated liver. A liver transplant may be done in patients with perihilar bile duct cancer. If the person has to wait for a donated liver, other treatment is given as needed.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. The following targeted therapies are being studied in patients with bile duct cancer that is locally advanced and cannot be removed by surgery or has spread to other parts of the body:
Immunotherapy
Immunotherapy is a treatment that uses the person’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer.
Immune checkpoint inhibitor therapy is a type of immunotherapy. Pembrolizumab is a immune checkpoint inhibitor that may be used to treat bile duct cancer.
Clinical trials
A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. For some people, taking part in a clinical trial may be the best treatment choice.
To learn more about clinical trials, see Clinical Trials Information for Patients and Caregivers.
Treatment of resectable (localized) bile duct cancer
If the cancer has not spread and is in a place where surgery can be safely done, the tumor and some of the tissue around it will be removed. This lowers the chance of the cancer coming back. Chemotherapy with or without radiation therapy may be given after surgery.
Treatment of resectable intrahepatic bile duct cancer may include the following:
Treatment of resectable perihilar bile duct cancer may include the following:
Treatment of resectable distal bile duct cancer may include the following:
Adjuvant therapy for resectable bile duct cancer may include the following:
Treatment of unresectable bile duct cancer (including metastatic or recurrent disease)
Most people with bile duct cancer cannot have their cancer completely removed with surgery. This may be the case if the cancer has spread too far, the cancer is in a place that is too difficult to completely remove with surgery, or the patient is not healthy enough for surgery. Treatment may include the following:
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