This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. 

        

Treatment overview

In cancer care, different types of doctors often work together to create a patient's overall treatment plan that combines different types of treatments. This is called a multidisciplinary team.

Descriptions of the most common treatment options for bile duct cancer are listed below. Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, and the patient's preferences and overall health. 

Surgery

Surgery is the removal of the tumor and surrounding tissue during an operation. A surgical oncologist is a doctor who specializes in treating cancer using surgery. 

Due to the location and sensitivity of the bile duct area, surgery for bile duct cancer can be difficult. And, it has limitations based on how large the tumor is and where it has spread. For instance, metastatic cancer cannot be completely removed surgically, and additional treatment will be necessary.

Surgical treatment options for bile duct cancer include:

Surgical removal of the bile duct. If the tumor has not spread beyond the bile duct, removing the organ surgically may be the recommended treatment. Lymph nodes (tiny, bean-shaped organs that help fight infection) may also be removed during the surgery and checked for cancer.

Partial hepatectomy. If the cancer is near the liver, the surgeon will remove part of the liver. The remaining section of liver takes over the functions of the entire liver and can, in some cases, regrow to its normal size within a few weeks.

Whipple procedure. If the cancer is near the pancreas, this surgery may be recommended. This is an extensive operation where the surgeon removes part or all of the pancreas and part of the small intestine, bile duct, and stomach, and then reconnects the digestive tract and biliary system. An experienced surgeon should perform this procedure. 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; others (25% to 45%) have serious complications, such as bleeding, infection, or leaking of bile or pancreatic juices.

Liver transplantation. Complete removal of the liver (total hepatectomy) and bile ducts followed by transplantation of a donor liver has been used mainly in research settings (clinical trials) at some centers to treat early stage cholangiocarcinoma. This approach may include chemotherapy and/or radiation therapy prior to the transplantation. Patients must be very carefully selected for this approach, and it is not an option for many patients. There may also be a long waiting list and unpredictable waiting period for a liver to become available for transplantation.

Stent placement and surgical bypass

In some situations, surgery cannot completely remove the tumor. However, surgery can still help relieve symptoms of the cancer and improve the patient's quality of life.

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

In addition to surgery, a plastic or metal stent can be passed through the blockage either during the ERCP procedure or during a procedure similar to PTC. Although these procedures do not remove the tumor, they can relieve the side effects and people often experience long periods of time when all of their symptoms disappear and quality of life is much better. For both of these procedures, the doctor may insert the stent internally, so the person is not aware of it. Sometimes, this is not possible, and a tube will be passed through the liver to redirect the bile externally (outside the body) into a bag that will need regular changing. Some doctors suggest that in these situations people receive long-term antibiotics to guard against infection.

Radiation therapy

Radiation therapy uses high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time. 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. Occasionally, internal radiation therapy may be used for bile duct cancer.

Radiation therapy can be used for treatment or to control the symptoms and pain of advanced disease. 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.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells' ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

Efforts to improve chemotherapy by investigating new drugs or new combinations of drugs are being made through clinical trials. This is often how patients with bile duct cancer receive chemotherapy.

Chemotherapy may be used before surgery to shrink the tumor or when surgery is not an option. Recent evidence suggests 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. Other drugs that have been used to treat bile duct cancer include fluorouracil (5-FU, Adrucil) and doxorubicin (Adriamycin). Chemotherapy may also be given after surgery to help prevent a recurrence, although how well this approach works is being researched in clinical trials.

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

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. 

Palliative/supportive care

Cancer and its treatment often causes side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person's symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person with any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem, so it is addressed as quickly as possible. 

Recurrent bile duct cancer

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

A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the cancer will come back. While many remissions are permanent, it's important to talk with your doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. 

If the cancer does return 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). 

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as surgery, radiation therapy, and chemotherapy) but may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.

People with recurrent cancer often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. 

Metastatic bile duct cancer

If cancer has spread to another location in the body, it is called metastatic cancer. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan. 

Your health care team may recommend a treatment plan that includes a combination of surgery, radiation therapy, and chemotherapy. Supportive care will also be important to help relieve symptoms and side effects.

For many patients, a diagnosis of metastatic cancer can be very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.

If treatment fails

Recovery from cancer is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.

This diagnosis is stressful, and it may be difficult to discuss. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Palliative care given toward the end of a person's life is called hospice care. You and your family are encouraged to think about where you would be most comfortable: at home, in a hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families.

Coping with Side Effects

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for bile duct cancer are described in detail within the Treatment section. 

Before treatment begins, talk with your doctor about the possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with bile duct cancer. 

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. 

During and after treatment, be sure to tell the health care team about the side effects your experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. 

After Treatment

After treatment for bile duct cancer ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. 

People treated for bile duct cancer may need to visit the doctor regularly for several years because there is always a risk that the tumor will recur after treatment or that the person will develop another tumor. Researchers are studying ways to prevent both recurrences and the development of secondary or “new” tumors, but currently, there is no standard preventive treatment.

If the tumor was not removed by surgery and a stent or surgical bypass was used to relieve jaundice, the most important part of follow-up care is making sure the stent or bypass procedure still works to remove the blockage. If jaundice becomes worse (a deeper yellow), if a person becomes jaundiced again after the stent has originally cleared the blockage, or if a person develops a fever along with sweating, shaking, or abdominal pain, a doctor should be consulted immediately, as the stent may need changing or repositioning.

A possible long-term side effect of treatment for bile duct cancer is the development of strictures after any surgery to the biliary system (bile ducts and gallbladder). These are noncancerous scars that form slowly, but may narrow the bile ducts causing symptoms similar to those of the original bile duct cancer.

People treated for bile duct cancer are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based on your needs, physical abilities, and fitness level. 

Latest Research

Doctors are working to learn more about bile duct cancer, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you.

A major focus of bile duct cancer research is to find out if new drugs that work differently than chemotherapy (such as anti-angiogenics; see below) might make the current standard chemotherapy work better for advanced biliary tract cancer.

Anti-angiogenesis therapy. Anti-angiogenesis therapy is focused on stopping angiogenesis, which is the process of making new blood vessels. Because a tumor needs the nutrients delivered by blood vessels to grow and spread, the goal of anti-angiogenesis therapies is to “starve” the tumor. Anti-angiogenic agents being studied for bile duct cancer treatment include bevacizumab (Avastin) and sorafenib (Nexavar).

Epidermal growth factor receptor (EGFR) inhibitors. Researchers have found that drugs that block EGFR may be effective in stopping or slowing the growth of bile duct cancer. Drugs being studied in this category include cetuximab (Erbitux), erlotinib (Tarceva), and panitumumab (Vectibix).

Photodynamic therapy (PDT). After the doctor gives an inactive form of a drug, he or she uses a special light that is directed at the tumor in the bile duct through an endoscope in a procedure similar to ERCP. This causes a chemical change in the drug, activating it to kill the tumor cells in the area where the light is directed. PDT is currently used to relieve pain and symptoms, not to cure bile duct cancer. Doctors are also looking at whether PDT can reduce the risk of a stent becoming blocked by stopping the tumor from growing into it.

Immunotherapy. Immunotherapy (also called biologic therapy) is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to bolster, target, or restore immune system function.

Hyperthermia therapy. Hyperthermia therapy kills cancer cells by increasing body temperature; it also may make cancer cells more likely to be killed by radiation therapy and chemotherapy.

Radiosensitizers. Researchers are looking at radiosensitizers to treat bile duct cancer. Radiosensitizers are drugs that make tumor cells more likely to be killed by radiation therapy.

Genetics. Scientists are also researching the genetic progression of bile duct cancer, which is the process of how one genetic change turns into many genetic changes that cause a cell to become cancerous.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current bile duct cancer treatments in order to improve patients' comfort and quality of life.