Bile Duct Cancer (Cholangiocarcinoma) Treatment and Survival Rate

November 22, 2024

This page was reviewed under our medical and editorial policy by Laleh Melstrom, M.D., M.S., associate professor of surgery and immuno-oncology, Division of Surgical Oncology, Department of Surgery, City of Hope^® Cancer Center Duarte

Bile duct cancer, also known as cholangiocarcinoma, is treated with a variety of therapies — from medications to surgery to supportive care. The diverse treatment options are matched to each patient based on the cancer type and location, the stage of the disease (how far it has spread), treatment side effects and the patient’s overall health at the time of diagnosis.

The patient’s goals for treatment, such as symptom relief or managing disease progression, are also considered when the care team creates a bile duct cancer treatment plan.

Who Treats Bile Duct Cancer?

The patient’s care team may consist of the following experts:

• Medical oncologists, who stage and treat bile duct cancer
• Hepatologists, who are experts in diseases affecting the liver and bile ducts
• Gastroenterologists, who treat diseases of the gastrointestinal system, including some cancers
• Surgical oncologists, who perform surgery to treat cancer
• Pathologists, who evaluate biopsies, tissue and tumors as part of the diagnosis process
• Radiation oncologists, who use high-energy beams of radiation to treat cancer
• Radiologists, who use diagnostic radiology tools to diagnose bile duct cancer and evaluate whether cancer has spread
• Specialized pharmacies, which supply medications that are difficult to find elsewhere, often for hard-to-treat conditions
• Supportive medicine providers, who manage cancer- and treatment-related side effects and symptoms

Treatment for Bile Duct Cancer

Cholangiocarcinoma treatment may include one or a combination of therapies and surgical procedures. For cancers that are able to be fully removed using surgery (resectable cancers), the main treatment is an operation to take it out.

Additional treatments may include:

• Chemotherapy
• Radiation therapy
• Immunotherapy
• Targeted therapy
• Liver transplant
• Palliative care

When the cancer is unresectable, meaning it is unable to be fully removed using surgery, the following therapies may be recommended instead. Sometimes, surgery is also used to help relieve symptoms caused by a blocked bile duct in unresectable bile duct cancer.

Surgery

There are two places where cholangiocarcinoma starts:

• Inside the liver’s bile ducts, which is called intrahepatic bile duct cancer
• In certain bile ducts outside the liver, which is called extrahepatic bile duct cancer

In addition, there are two types of extrahepatic bile duct cancer, as listed below.

Perihilar bile duct cancer: Also called Klatskin tumors, this type of cancer begins in the hilum, the region where the left and right bile ducts leave the liver as they connect to form the common hepatic duct.

Distal bile duct cancer: This type of cancer begins in the common bile duct near the small intestine.

Cholangiocarcinoma surgery is the first treatment option used to treat resectable (removable) bile duct cancer. The type of surgery performed depends on where the cholangiocarcinoma starts.

Partial hepatectomy: This is surgery to remove the part of the liver where cancer is found. It is used to treat intrahepatic bile duct cancer.

Hepatic lobectomy: Sometimes, intrahepatic bile duct cancer has spread throughout an entire lobe of the liver and surgery is performed to remove that lobe. The liver may still function normally after a hepatic lobectomy performed by an experienced surgeon.

Perihilar bile duct cancer surgery: This type of bile duct cancer surgery includes the removal of the bile duct where cancer is found, as well as a portion of the liver, the gallbladder and sometimes parts of the small intestine and pancreas. A surgery called porta hepatis lymphadenectomy is also performed, which is the removal of the lymph nodes around the hilum. A Roux-en-Y hepaticojejunostomy, a type of gastric bypass surgery, is then performed, in which the remaining bile ducts are connected to the small intestine so that the digestive bile is still able to reach it.

Whipple procedure: This is an extensive surgical procedure to remove the bile duct where cancer is found, the surrounding lymph nodes (lymphadenectomy) and parts of the small intestine and pancreas. It is used for distal bile duct cancer.

Prior to each of these procedures, the doctor may perform exploratory surgery to discover areas where the cancer has spread that may not appear on imaging tests. This type of diagnostic surgery is called laparoscopy or laparoscopic surgery, and it is also used to plan the second surgical procedure if the cancer is resectable. When possible, the main surgery may be performed laparoscopically. This means that only tiny incisions are made to insert tools into the body for the procedure. In addition, robotic surgery may be used for some bile cancer resections because it is less invasive than open surgery (which is surgery that uses a large cut to the abdomen to allow the surgeon access to the bile duct). Robotic surgery utilizes a robotic arm with attached surgical tools, which is manipulated by the surgeon. These tools are inserted through smaller incisions made in the abdominal wall.

Chemotherapy

Chemotherapy refers to drugs that destroy cancer cells. It is used to treat bile duct cancer both before surgery (neoadjuvant chemotherapy) and after surgery (adjuvant chemotherapy).

Chemotherapy may also be used with other therapies or by itself to treat bile duct cancer that has not been surgically removed or that has returned. In addition, it is given to help reduce certain symptoms of cancer, such as to shrink a tumor that is touching a nerve in order to reduce a patient’s pain levels.

Two main types of chemotherapy are used to treat bile duct cancer.

Systemic chemotherapy is given by intravenous (IV) vein infusion or by mouth (pill or capsule), usually in cycles that include a period of treatment followed by a period of recovery. These drugs destroy bile duct cancer cells throughout the body and include drug combinations such as:

• Gemzar^® (gemcitabine) and Platinol^® (cisplatin)
• Gemcitabine and Xeloda^® (capecitabine)
• GEMOX (gemcitabine and oxaliplatin)
• XELOX (capecitabine and oxaliplatin)

Regional chemotherapy is chemotherapy that is placed in one area of the body to destroy cancer cells there, such as in an organ, body cavity or specific blood vessel. For bile duct cancer, regional chemotherapy treatments include hepatic artery infusion and transarterial chemoembolization.

Hepatic artery infusion (HAI): The main artery that supplies blood to the liver — and to bile duct tumors — is called the hepatic artery. To help destroy the bile duct tumors, a catheter is surgically placed right into the hepatic artery and chemotherapy drugs are inserted into it. This directs more medication to the tumor itself. The liver filters out the remaining drugs, which helps reduce chemotherapy’s impacts on the rest of the body. HAI may be performed if the cancer is unresectable.

Transarterial chemoembolization (TACE): These are chemotherapy beads placed (via a catheter) directly into an artery that supplies a bile duct tumor with blood. They wedge into the artery to block the supply of blood to the tumor, while also releasing drugs that help destroy the cancer. TACE is used for bile duct tumors that surgeons are unable to remove.

Because chemotherapy drugs target and destroy cells in the body that divide (reproduce) quickly, they not only impact cancer cells, but also noncancerous cells that tend to divide quickly, such as healthy bone marrow and hair follicle cells. This may cause the following side effects:

• Nausea and vomiting
• Loss of appetite
• Diarrhea
• Increased infections
• Fatigue
• Bruising and bleeding
• Mouth sores
• Hair loss
• Pain, numbness or tingling from nerve damage, especially in the feet and hands

Some medicines and other therapies may help reduce chemotherapy side effects, which the doctor may recommend based on the specific effects each patient experiences.

Radiation Therapy

Radiation therapy is the use of high-energy X-rays, protons and other particles to destroy cancer cells.

Two main types of radiation therapy are in use. External beam radiation therapy (EBRT) is directed at cancer cells by a machine outside a person’s body. A few types of EBRT treatments are used to treat bile duct cancer, including the following.

3D conformal radiation therapy (3D-CRT): This therapy sends radiation beams from different angles that are fitted to the tumor’s shape in order to reduce injury to surrounding (healthy) tissues.

Intensity-modulated radiation therapy (IMRT): This is similar to 3D-CRT, but in addition to conforming the radiation beams to the tumor’s shape, their intensity may also be adjusted.

Stereotactic body radiotherapy: This type of therapy is high-dose IMRT or 3D-CRT radiation therapy given in a couple of sessions, usually over the course of a week, rather than the typical five days a week over three to six weeks.

A few techniques are being studied to improve EBRT’s successfulness. For instance, drugs called radiosensitizers may help make cancer cells easier to destroy with radiation. Heating body tissues in a process called hyperthermia therapy may also make cancer cells more sensitive to radiation treatment.

The potential side effects of EBRT include:

• Low blood cell counts
• Nausea and vomiting
• Diarrhea
• Skin redness, blisters or peeling
• Hair loss
• Fatigue

The second main type of radiation therapy is internal beam radiation therapy (IBRT), or brachytherapy. It is often used as a palliative treatment (to improve quality of life) for bile duct cancer and includes radioactive substances, such as seeds (pellets), wires, catheters or needles, that are inserted near or into the bile duct to destroy cancer cells.

Targeted Therapy

Changes (mutations) may form in certain cell components, and these changes sometimes trigger the production of cancer cells. Targeted therapy drugs work to address these changes in order to help stop or slow down bile cancer growth and spread. For example, IDH1 inhibitors block a mutation in the IDH1 gene that helps bile duct cancer cells mature.

Targeted therapy drug side effects will vary based on the drug being taken. These may range from milder side effects — such as diarrhea, nausea and joint pain — to more serious side effects, such as organ damage or severe allergic reactions. Patients should check with their doctor to find out what potential impacts they should expect from each prescribed medication.

Immunotherapy 

Immunotherapy is a treatment that assists the body’s own immune system in fighting cancer.

For bile duct cancer, the checkpoint inhibitor PD-1 is a key immunotherapy treatment. A checkpoint is a protein on an immune system cell that normally stops the body from attacking its own cells. However, cancer cells may use these checkpoints to thwart an immune system attack. Pembrolizumab (Keytruda®) helps suppress the PD-1 checkpoint on immune system T cells so that the T cells attack bile duct cancer cells.

Side effects of PD-1 inhibitors may include:

• Nausea
• Loss of appetite
• Weakness and fatigue
• Skin rash or itching
• Diarrhea
• Constipation
• Muscle or joint discomfort
• Cough
• Shortness of breath

Liver Transplant

If a patient is diagnosed with perihilar bile duct cancer, a liver transplant may be recommended. Here, the surgical team removes the entire liver and replaces it with a healthy liver from an organ donor. While the patient awaits the donated liver, they often receive other treatments, such as chemotherapy, immunotherapy or radiation and targeted therapies.

Palliative Treatment

When treatment is given to help people manage bile duct cancer symptoms and make a person more comfortable, it is known as palliative therapy. The goal is not to control the disease but to help people feel better.

There are a number of palliative treatments for bile duct cancer, including chemotherapy and radiation therapy treatments to reduce the effects of tumors. Medications to reduce symptoms like pain or nausea may also be given to improve the patient’s quality of life.

Procedures used for cholangiocarcinoma palliative care include the following.

Biliary stent or catheter placement: If a tumor is blocking the bile duct, a plastic or metal tube (stent) may be placed into the duct to allow for the flow of bile into the small intestine. Other times, a long flexible tube called a catheter is placed through an opening made by a surgeon in the patient’s abdomen. One end is put into the bile duct before the blockage, while the other sticks out of the opening and drains the bile into a bag. Both of these procedures help reduce symptoms of the blockage so the patient is more comfortable.

Biliary bypass: To bypass a tumor blockage in the bile duct and allow for the drainage of digestive bile into the small intestine, the surgeon disconnects the bile duct before the tumor and attaches it to a section of the bile duct (or small intestine) beyond the blockage.

Photodynamic therapy: Here, a drug that is activated by light and gathers in cancer tumors is injected into a vein. Next, a laser light that is attached to an endoscope (a long, flexible instrument with a camera) is inserted down the throat. The light activates the drug, which destroys the cancer cells.

Tumor ablation: A probe is guided into a small opening in the body and then into the tumor using an imaging scan such as an ultrasound. The tumor is destroyed (ablated) by heating the probe’s tip in a procedure known as radiofrequency ablation (RFA). Sometimes, the tip is frozen, which is called cryotherapy.

Patients may also help guide their palliative care through lifestyle decisions, including eating healthy and avoiding habits like smoking or drinking alcohol. Foods to avoid with bile duct cancer include fatty foods, since the body may not be able to digest these, especially if the cancer tumor is blocking the flow of (fat-digesting) bile.

Bile Duct Cancer Survival Rate

The proportion of people with bile duct cancer still alive after five years or more, compared to the overall population, is known as the five-year relative survival rate.

For people diagnosed with bile duct cancer between 2012 and 2018, the American Cancer Society reports that the five-year relative survival rate was:

• 9% for intrahepatic bile duct cancer
• 11% for extrahepatic bile duct cancer

This bile duct cancer prognosis highlights the need for continued research into treatments to better manage and control the disease. Targeted therapies that have been developed in recent years have shown promise for improving people’s cholangiocarcinoma prognosis when combined with other treatments, and more treatments are being researched and evaluated in clinical trials.

Bile Duct Cancer Treatment at City of Hope

Every patient is different, with different needs. City of Hope treats patients as valued individuals throughout their fight with cancer — getting to know their health, values and needs. Our entire team works together to bring state-of-the-art surgical, radiation, drug and other therapy options to the table, tailored to each person.

At City of Hope, bile duct cancer patients have support every step of the way, during treatment and long after, from an expert medical team focused on helping them and their loved ones manage the treatment journey and recovery stage.

When possible, City of Hope surgeons take minimally invasive approaches to remove bile duct cancer, such as laparoscopic surgery and robotically assisted surgery, giving the surgeon superior visualization and greater control at the tumor site.

At City of Hope, we use precise radiation delivery techniques. Using advanced imaging techniques, our radiation team can accurately locate and target a bile duct tumor for treatment, ensuring maximum impact against cancer cells while minimizing exposure to surrounding normal tissues.