Bile Duct Cancer (Cholangiocarcinoma): Types of Treatment

Approved by the Cancer.Net Editorial Board, 09/2023

ON THIS PAGE: You will learn about the different treatments doctors use for people with bile duct cancer. Use the menu to see other pages.

This section explains the types of treatments, also known as therapies, that are the standard of care for bile duct cancer. “Standard of care” means the best treatments known. Information in this section is based on medical standards of care for bile duct cancer in the United States. Treatment options can vary from one place to another.

When making treatment plan decisions, you are encouraged to discuss with your doctor whether clinical trials offer additional options to consider. A clinical trial is a research study that tests a new approach to treatment. Doctors learn through clinical trials whether a new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Clinical trials are an option for all stages of cancer. Your doctor can help you consider all your treatment options. Learn more about clinical trials in the About Clinical Trials and Latest Research sections of this guide.

How bile duct cancer is treated

In cancer care, different types of doctors who specialize in cancer, called oncologists, often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team. Cancer care teams include other health care professionals, such as physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, dietitians, physical therapists, occupational therapists, and others. Learn more about the clinicians who provide cancer care.

Treatment options and recommendations depend on several factors, including:

  • The type and stage of cancer

  • Possible side effects

  • The person’s preferences and overall health

Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. These types of conversations are called “shared decision-making.” Shared decision-making is when you and your doctors work together to choose treatments that fit the goals of your care. Shared decision-making is important for bile duct cancer because there are different treatment options. Learn more about making treatment decisions.

The common types of treatments used for bile duct cancer are described below. Your care plan also includes treatment for symptoms and side effects, an important part of cancer care.

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Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue, called a margin, during an operation. A surgical oncologist is a doctor who specializes in treating cancer using surgery. Surgery to treat bile duct cancer may also be done by a hepatobiliary surgeon, who has special training in treating the liver and bile ducts.

The location and sensitivity of the bile duct area make surgery difficult. The surgery’s effectiveness may also be limited by the tumor's size and where it has spread (see the Stages section). For instance, surgery cannot completely remove metastatic cancer (see below), so additional treatment is necessary in those cases. Talk with your surgeon about what to expect with your specific surgery. If surgery is not an option, the tumor may be called "inoperable" or "unresectable."  In this situation, your doctor will recommend other treatment options. 

Common surgical treatment options for bile duct cancer include:

  • Surgical removal of the bile duct. The surgeon removes the entire organ. This is a treatment option if the tumor has not spread beyond the bile duct. The surgery may also include removal of lymph nodes to check for cancer. Lymph nodes are small, bean-shaped organs that help fight infection.

  • Partial hepatectomy. If the cancer is near the liver, the surgeon will remove part of the liver. The remaining section of the liver takes over the functions of the entire liver. In some cases, it grows back to its normal size within a few weeks.

  • Whipple procedure. The Whipple procedure is an extensive type of surgery that may be recommended if the cancer is near the pancreas. The surgeon removes part or all of the pancreas and part of the small intestine, bile duct, and stomach. The surgeon then reconnects the digestive tract and biliary system, which includes the bile ducts and gallbladder. To maintain the flow of bile, the remaining part of the bile duct is connected to the small intestine. About 5% to 10% of people do not survive this complicated operation. Meanwhile, 25% to 45% have serious complications, such as bleeding, infection, or leaking of bile or pancreatic juices. An experienced surgeon should always perform this procedure.

  • Liver transplantation. The surgeon completely removes the liver and bile ducts. Surgery to remove the liver is called a total hepatectomy. The surgeon then transplants a donor liver. However, bile duct cancer tends to recur very quickly after transplantation. As a result, this procedure is rarely used.

Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have. Learn more about the basics of cancer surgery.

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Radiation therapy

Radiation therapy uses high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period.

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation therapy is given using implants, it is called internal radiation therapy, or brachytherapy. Internal radiation therapy may be used occasionally for bile duct cancer. Some cancer centers may use a specific type of radiation therapy called radioembolization. During this treatment, a doctor places radioactive beads into the artery that supplies the tumor with blood. The beads deliver radiation directly into the tumor when they become trapped in the small blood vessels of the tumor.

Radiation therapy can be used for treatment of the cancer or to control the symptoms and pain of advanced disease as part of palliative care. It may also be combined with chemotherapy (see below), which is a combination treatment called chemoradiotherapy. In some situations, chemoradiotherapy may be given after surgery to destroy any remaining cancer cells. Talk with your health care team about whether this is a good treatment option for you. Learn more about the American Society of Clinical Oncology's (ASCO) recommendations for treating bile duct cancer with chemoradiotherapy on a separate website.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. Learn more about the basics of radiation therapy.

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Therapies using medication

The treatment plan may include medications to destroy cancer cells. Medication may be given through the bloodstream to reach cancer cells throughout the body. When a drug is given this way, it is called systemic therapy. Medication may also be given locally, which is when the medication is applied directly to the cancer or kept in a single part of the body.

This treatment is generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.

Medications are often given through an intravenous (IV) tube placed into a vein using a needle or as a pill or capsule that is swallowed (orally). If you are given oral medications to take at home, be sure to ask your health care team about how to safely store and handle them.

The types of medications used for bile duct cancer include:

  • Chemotherapy

  • Targeted therapy

  • Immunotherapy

Each of these types of therapies is discussed below in more detail. A person may receive 1 type of medication at a time or a combination of medications given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy.

The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. It is also important to let your doctor know if you are taking any other prescription or over-the-counter medications or supplements. Herbs, supplements, and other drugs can interact with cancer medications, causing unwanted side effects or reduced effectiveness. Learn more about your prescriptions by using searchable drug databases.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by keeping the cancer cells from growing, dividing, and making more cells.

A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or a combination of different drugs given at the same time.

Researchers are working to improve chemotherapy by studying new drugs or new combinations of drugs through clinical trials. People with bile duct cancer may receive chemotherapy by participating in a clinical trial.

Chemotherapy may be used before surgery to shrink the tumor. It may also be used when surgery is not an option. Research has suggested that the combination of cisplatin (Platinol) and gemcitabine (Gemzar) can lengthen the lives of patients who have bile duct cancer that cannot be removed by surgery.

Chemotherapy should be given after surgery to help prevent a recurrence. ASCO recommends capecitabine (Xeloda) for 6 months after surgery. Additionally, people with hilar cholangiocarcinoma and extrahepatic cholangiocarcinoma may also receive chemoradiotherapy after surgery and capecitabine to destroy remaining cancer cells when there is a positive margin that can be seen with a microscope. A positive margin means that there are cancer cells around the area where the tumor was removed (see "Radiation therapy," above, for more on the ASCO recommendations).

Other drugs that have been used to treat bile duct cancer include fluorouracil (5-FU, Adrucil) and paclitaxel (Taxol). However, bile duct cancers are resistant to many types of chemotherapy, which makes clinical trials much more important for treating this disease.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away after treatment is finished.

Learn more about the basics of chemotherapy.

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Targeted therapy

Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells and limits damage to healthy cells.

Not all tumors of the same type or tissue origin have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. This helps doctors better match each patient with the most effective treatment whenever possible. In addition, research studies continue to find out more about specific molecular targets and new treatments directed at them. Learn more about the basics of targeted treatments.

The U.S. Food and Drug Administration (FDA) has approved both pemigatinib (Pemazyre) and infigratinib (Truseltiq) for people with previously treated, unresectable locally advanced or metastatic cholangiocarcinoma with an FGFR2 gene fusion, which occurs in about 10% to 15% of patients. Unresectable means that surgery is not an option. A gene fusion is when 2 genes that used to be separate have joined together and don't function normally as a result. The FGFR2 protein, which is made by the FGFR2 gene, helps cancer grow and spread when it has undergone fusion and becomes overactive. Pemigatinib and infigratinib both bind to the FGFR2 protein to shut down the hyperactivity of FGFR2 and slow down cancer cells with that protein from growing and spreading. Pemigatinib is an oral medication that is given in 21-day cycles. It is given once a day for 14 days followed by 7 days off. Infigratinib is an oral medication that is given once a day for 21 days followed by 7 days off. It should be noted that pemigatinib and infigratinib have not been compared head-to-head in terms of effectiveness.

In 2021, the FDA approved ivosidenib (Tibsovo) for people with previously treated locally advanced or metastatic cholangiocarcinoma with an isocitrate dehydrogenase-1 (IDH1) genetic mutation, which occurs in about 15% of people with this type of cancer. A mutation in the IDH1 gene helps cancer grow, and ivosidenib works by targeting and blocking this gene to slow down cancer growth and spread. Ivosidenib is an oral medication that is given once a day.

In 2022, the FDA approved dabrafenib (Tafinlar) in combination with trametinib (Mekinist) for the treatment of people with biliary tract cancer with a BRAF V600E mutation that is either metastatic or that cannot be removed with surgery. BRAF V600E mutations occur in about 3% of people with cholangiocarcinoma. To receive the combination, a patient's cancer must have either gotten worse following previous treatment or there must be no other satisfactory treatment options available. A BRAF V600 mutation is a specific change in the tumor’s BRAF gene. Dabrafenib is a targeted therapy that works by targeting the BRAF mutation, and trametinib is a targeted therapy that targets the MEK protein, which is involved in cancer growth and survival. Dabrafenib is given as an oral medication twice daily, and trametinib is given as an oral medication once daily.

Like other treatments, targeted therapies can cause side effects, so it is important that your doctor matches your tumor to the best possible treatment and dose. Talk with your doctor about possible side effects for a specific medication and how they can be managed.

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Immunotherapy

Immunotherapy uses the body's natural defenses to fight cancer by improving your immune system’s ability to attack cancer cells.

Durvalumab (Imfinzi) or pembrolizumab (Keytruda) may be used in combination with the chemotherapy drugs gemcitabine and cisplatin for people with locally advanced or metastatic biliary tract cancer, including those with bile duct cancer and gallbladder cancer. Durvalumab and pembrolizumab are a type of immunotherapy called an immune checkpoint inhibitor. An immune checkpoint is a protein that can stop the body’s immune system from responding to cancer cells. Durvalumab and pembrolizumab work by targeting and blocking the immune checkpoint PD-1, which then allows the immune system to find and attack cancer cells.

Pembrolizumab and another immune checkpoint inhibitor, nivolumab (Opdivo), are also approved for all microsatellite instability-high (MSI-H) tumors, including for MSI-H bile duct cancer, which accounts for about 1% of biliary tract cancers. MSI is a genomic marker that indicates a flaw in a cell’s ability to repair damaged DNA, which can lead to an increase in genetic changes, or mutations. These mutations produce abnormal proteins on the tumor cells that allow immune cells to find and attack the tumor more easily.

Different types of immunotherapy can cause different side effects. Common side effects include skin reactions, flu-like symptoms, diarrhea, and weight changes. Talk with your doctor about possible side effects for the immunotherapy recommended for you. Learn more about the basics of immunotherapy.

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Physical, emotional, social, and financial effects of cancer

Cancer and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative and supportive care. It is an important part of your care that is included along with treatments intended to slow, stop, or eliminate the cancer.

Palliative and supportive care focuses on improving how you feel during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of cancer, may receive this type of care. And it often works best when it is started right after a cancer diagnosis. People who receive palliative and supportive care along with treatment for the cancer often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. You may also receive palliative treatments, such as chemotherapy, surgery, or radiation therapy, to improve symptoms. For instance, palliative surgical options may include stent placement and surgical bypass (see below). 

Before treatment begins, talk with your doctor about the goals of each treatment in the recommended treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative and supportive care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.

Cancer care is often expensive, and navigating health insurance can be difficult. Ask your doctor or another member of your health care team about talking with a financial navigator or counselor who may be able to help with your financial concerns.

During treatment, your health care team may ask you to answer questions about your symptoms and side effects and to describe each problem. Be sure to tell the health care team if you are experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative and supportive care in a separate section of this website.

Stent placement and surgical bypass

In some cases, surgery cannot completely remove the bile duct tumor, but it can still help relieve symptoms and improve the person’s quality of life.

In this situation, a surgeon may be able to bypass the blocked area. This involves connecting a part of the bile duct located before the blockage to a part of the small intestine located beyond the blockage. During this procedure, the surgeon may insert a plastic or metal tube into the bile duct to keep it open. This tube is called a stent.

In addition, a plastic or metal stent may be used to pass through the blockage during an endoscopic retrograde cholangiopancreatography (ERCP) procedure or a procedure similar to percutaneous transhepatic cholangiography (PTC). Both of these procedures are described in the Diagnosis section earlier in this guide. These procedures do not remove the tumor. However, they can relieve side effects, and people often experience long periods when all symptoms disappear and their quality of life improves after these procedures.

For both of these procedures, the doctor may insert the stent internally. However, sometimes this is not possible. In those cases, a tube passes through the liver to redirect the bile outside of the body and into a bag. The bag needs to be changed regularly. Some doctors suggest that these patients receive long-term antibiotics to protect against infection. If this is recommended for you, talk with your health care team about maintaining the bag and watching for signs of infection.

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Metastatic bile duct cancer

If cancer spreads to another part of the body from where it started, doctors call it metastatic cancer. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Clinical trials might also be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your chosen treatment plan.

Your treatment plan may include a combination of surgery, radiation therapy, and therapies using medication. Palliative and supportive care will also be important to help relieve symptoms and side effects.

For many people, a diagnosis of metastatic cancer is very stressful and difficult. You and your family are encouraged to talk about how you feel with doctors, nurses, social workers, or other members of your health care team. It may also be helpful to talk with other patients, such as through a support group or other peer support program.

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Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.

A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the cancer returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.

If the cancer returns after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

If a recurrence happens, a new cycle of testing will begin to learn as much as possible about it. After this testing is done, you and your doctor will talk about the treatment options. Often the treatment plan will include the treatments described above, such as surgery, radiation therapy, and/or chemotherapy, but they may be used in a different combination or given at a different pace. Your doctor may suggest clinical trials that are studying new ways to treat recurrent bile duct cancer. Whichever treatment plan you choose, palliative and supportive care will be important for relieving symptoms and side effects.

People with recurrent cancer sometimes experience emotions such as disbelief or fear. You are encouraged to talk with your health care team about these feelings and ask about support services to help you cope. Learn more about dealing with cancer recurrence.

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If treatment does not work

Recovery from bile duct cancer is not always possible. If the cancer cannot be cured or controlled, the disease may be called advanced or terminal.

This diagnosis is stressful, and for some people, advanced cancer is difficult to discuss. However, it is important to have open and honest conversations with your health care team to express your feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help. Making sure a person is physically comfortable, free from pain, and emotionally supported is extremely important.

Planning for your future care and putting your wishes in writing is important, especially at this stage of disease. Then, your health care team and loved ones will know what you want, even if you are unable to make these decisions. Learn more about putting your health care wishes in writing.

People who have advanced cancer and who are expected to live less than 6 months may want to consider hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to talk with your doctor or a member of your palliative care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.

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The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with cancer. Use the menu to choose a different section to read in this guide.