What Is Bile Duct Cancer? Symptoms, Causes, Treatments, and More

Bile duct cancer, or cholangiocarcinoma, is a rare type of cancer that starts in the bile ducts. Bile ducts are small tubes in your body that carry bile (digestive fluids) from your liver and gallbladder to your small intestine. Once in the small intestine, bile helps you digest fats from the foods you eat.

Though bile duct cancer can affect anyone, it’s more common in individuals older than age 65, according to the National Institutes of Health (NIH).

Bile duct cancer is often difficult to treat because most people don’t get a diagnosis until the cancer has already spread to other areas of the body. However, recent medical advances and newer treatment options have improved the outlook for people with this type of cancer.

Doctors categorize bile duct cancer into three types, depending on where the cancer is located:

• Intrahepatic Cholangiocarcinoma This type starts in bile ducts in the liver. It’s sometimes confused with cancer that starts in the liver (called hepatocellular carcinoma).
• Hilar Cholangiocarcinoma The variety occurs in the bile ducts just outside the liver. It’s also called perihilar cholangiocarcinoma.
• Distal Cholangiocarcinoma This type is located farther down in bile ducts near the small intestine.

The hilar and distal forms of bile duct cancer, which are both found outside the liver, are sometimes grouped together as extrahepatic cholangiocarcinoma.

Most bile duct cancers are cholangiocarcinomas, which begin in the gland cells that line the inside of the ducts, according to the American Cancer Society.

However, there are also some less common types of bile duct cancers, including:

• Sarcomas
• Lymphomas
• Small cell cancers

Bile duct cancer doesn’t usually cause any obvious symptoms in its early stages. Symptoms are more likely to occur as the tumor progresses and bile ducts become blocked.

Some possible signs and symptoms of bile duct cancer include:

• Jaundice (yellowing of the skin or whites of the eyes)
• Itchy skin
• White- or pale-colored stools
• Fatigue
• Nausea or vomiting
• Abdominal pain on the right side of your body
• Fever
• Night sweats
• Unexplained weight loss
• Dark-colored urine

If your doctor suspects you might have bile duct cancer, you may undergo different tests, including the following:

• Liver Function Test This is a blood test that measures certain substances that the liver releases into the blood.
• Tumor Marker Test It checks your blood for levels of carbohydrate antigen 19-9, a protein that bile duct cancer cells overproduce.
• Endoscopic Retrograde Cholangiopancreatography Doctors insert a thin tube with a camera down the digestive tract and into the small intestine. They use the camera to view the spot where the bile ducts meet the small intestine. Doctors sometimes also inject a dye into the bile ducts to better visualize them.
• Imaging Tests Your provider may recommend other imaging procedures to check for signs of cancer. Some common tests for diagnosing bile duct cancer include ultrasound, computed tomography, positron emission tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography.
• Biopsy A biopsy is a procedure to remove a small piece of tissue to examine under a microscope in the lab.

If your doctor confirms a diagnosis of bile duct cancer, the next step is to stage the cancer. The stage describes how advanced the cancer is and helps your provider develop a treatment plan. Stages are designated by a number between 0 and 4. The higher the number, the more advanced the cancer.

Doctors stage bile duct cancer by evaluating the size of the tumor and determining whether it’s spread to other areas, including blood vessels, lymph nodes, organs near the bile ducts (such as the liver or gallbladder), or distant organs (such as the lungs, bones, or abdominal cavity), according to the Cleveland Clinic.

Certain diseases that affect the liver and bile ducts may increase your risk of developing bile duct cancer. Those include:

• Primary sclerosing cholangitis (hardening and scarring of the bile ducts)
• Bile duct stones (small stones that can cause inflammation)
• Choledochal cyst disease (bile duct cysts)
• Cirrhosis (scarring of the liver)
• Hepatitis B virus
• Hepatitis C virus
• Abnormalities where the bile and pancreatic duct meet
• Clonorchiasis (a parasite in the liver)
• Nonalcoholic fatty liver disease (a buildup of fat in the liver cells that’s not due to alcohol use)
• Polycystic liver disease (a rare, genetic disease of the liver)
• Caroli syndrome (an abnormality of the intrahepatic bile ducts that is present at birth)

Other factors that many elevate your chances of having bile duct cancer include:

• Being Hispanic American
• Living in an area where liver parasites are common
• Having type 1 or type 2 diabetes
• Being older than 50 years of age
• Being obese
• Having ulcerative colitis or inflammatory bowel disease
• Being exposed to toxins, such as radon, asbestos, or thorium dioxide
• Having a family history of bile duct cancer
• Being a current or former smoker
• Having an alcohol use disorder
• Having human immunodeficiency virus (HIV)

Complications of bile duct cancer generally happen when the tumor blocks a bile duct. This can cause problems such as:

• Jaundice
• Infection
• Liver dysfunction or liver failure

The outlook for people with bile duct cancer depends on the cancer’s stage, the location of the tumor, the patient’s overall health, the treatments they receive, and other factors.

According to the American Cancer Society, the five-year survival rate for all stages of the disease is 10 percent for extrahepatic bile duct cancers (those that start outside the liver) and 9 percent for intrahepatic bile duct cancers (those that start in the liver). It’s important to know that these statistics are only estimates based on existing data, and they won’t predict what will happen in every case. Additionally, survival rates may be higher or lower depending on the stage of the disease.

The location, size, and stage of your cancer will determine your treatment options. Doctors usually use a combination of different therapies to treat bile duct cancer.

Surgery

Surgery is an option for bile duct cancers that have been caught early and haven’t spread to other areas of the body. Surgeons will try to remove as much of the cancer as possible. If the cancer is more advanced, they may have to also remove liver tissue, pancreatic tissue, and/or lymph nodes.

In some cases, doctors might recommend a liver transplant. This involves removing the liver and replacing it with a donor liver. This procedure can sometimes cure people with very specific types of bile duct cancer. However, finding a donor is often challenging, and very few people are candidates for a liver transplant, according to the American Cancer Society.

Chemotherapy

Chemotherapy, which uses drugs to kill cancer cells, may be an option for certain people with bile duct cancer. Common chemotherapy regimens, according to the National Cancer Institute, include:

• Gemcitabine (Gemzar) and cisplatin (Platinol)
• XELOX: capecitabine (Xeloda) and oxaliplatin (Eloxatin)
• GEMOX: gemcitabine (Gemzar) and oxaliplatin (Eloxatin)
• Gemcitabine (Gemzar) and capecitabine (Xeloda)

Sometimes, doctors perform the following special procedures to deliver chemotherapy directly to the affected bile duct:

• Transarterial Chemoembolization Tiny beads are placed into the blood vessels to stop blood from reaching the tumor. The beads also release chemotherapy drugs to shrink the tumor.
• Hepatic Artery Infusion This procedure involves using a surgically implanted pump to inject chemo directly into the main artery that supplies blood to the liver.

Radiation

Radiation therapy uses beams of energy to kill cancer cells in the body. According to the American Cancer Society, radiation isn’t a common treatment for bile duct cancer, but it can be used in some situations, such as before or after surgery or for advanced cancers. The treatment may be delivered using the following methods:

• External Beam Radiation A machine directs radiation beams at your body.
• Brachytherapy Radioactive materials are placed inside your body near the cancer site.

Targeted Therapy

Targeted therapy involves using treatments that block specific abnormalities in cancer cells. These therapies may be suitable for people with bile duct cancer caused by a faulty gene that allows proteins to develop on their cancer cells. According to the American Cancer Society, some targeted treatments used for bile duct cancer are:

• FGFR2 Inhibitors These block the abnormal FGFR2 protein in bile duct cancer cells. FGFR2 inhibitors include pemigatinib (Pemazyre), infigratinib (Truseltiq), and futibatinib (Lytgobi).
• IDH1 Inhibitor The medicine ivosidenib (Tibsovo) is an IDH1 inhibitor. It works by blocking the abnormal IDH1 protein.

Immunotherapy

Immunotherapy employs your body’s own immune system to fight cancer. It can be an option for advanced bile duct cancers when other treatments have failed.

The immunotherapy medicine pembrolizumab (Keytruda) may help treat some cases of bile duct cancer, according to the National Cancer Institute.

In September of 2022, the U.S. Food and Drug Administration also approved the immunotherapy drug durvalumab (Imfinzi) in combination with the chemotherapy regimen of gemcitabine (Gemzar) and cisplatin (Platinol) for people with advanced bile duct cancer. The approval came after a clinical trial found that adding the immunotherapy treatment extended survival by about six weeks in people with advanced biliary tract cancers.

Ablation

Bile duct cancer that forms in the liver may be treated with ablation methods, such as:

• Cryotherapy This involves using extreme cold to freeze abnormal tissue.
• Radiofrequency Ablation This method uses heat to destroy cancer cells.

While these treatments may help control tumors, nearly all will start to grow back in the future, according to the American Cancer Society.

Palliative Treatments

Palliative treatments are used to help relieve symptoms of advanced bile duct cancer, rather than cure the disease. Chemotherapy, radiation, ablation, and surgery may all be used to provide palliative care. Other approaches may include:

• Biliary Stent or Catheter If cancer blocks a bile duct, doctors can place a small stent or catheter into the duct to keep it open.
• Biliary Bypass Surgeons create a bypass around a blocked bile duct by connected parts of the duct or the intestine.
• Photodynamic Therapy A light-activated medicine that collects in cancer cells is injected into your vein. A few days later, doctors use a special laser to activate the drug and kill cancer cells.
• Alcohol Injection Doctors inject affected nerves with alcohol to help lessen pain.

Clinical Trials

Clinical trials may be an option if you have bile duct cancer. These research studies test experimental therapies that aren’t yet available to the public. If you participate in a clinical trial, you may receive a novel treatment that wouldn’t be offered to you otherwise.

You can lower your risk of bile duct cancer by:

• Quitting smoking
• Avoiding certain viruses, such as HIV, hepatitis B, and hepatitis C
• Getting vaccinated against hepatitis B
• Limiting alcohol consumption
• Maintaining a healthy weight
• Avoiding being around environmental toxins

Bile duct cancer is rare. Only about 8,000 people in the United States develop it each year, according to the American Cancer Society. However, researchers believe the number of cases may be higher because bile duct cancer is hard to detect and may be misdiagnosed.

The average age of diagnosis in the United States is 70 for intrahepatic bile duct cancer and 72 for extrahepatic bile duct cancer. The cancer affects slightly more men than women, according to the NIH.

Bile duct cancer is more common in Southeast Asia, where parasite infections that cause the cancer are more widespread.

The American Cancer Society says the risk of bile duct cancer in the United States is highest among Hispanics. Hispanic men and women are 1.7 times more likely to die of intrahepatic bile duct cancer than whites, according to the U.S. Department of Health and Human Services Office of Minority Health. Researchers are studying this correlation to better understand why it exists.

Some conditions closely related to bile duct cancer include:

• Liver cancer
• Gallbladder cancer
• Cirrhosis
• Nonalcoholic fatty liver disease
• Hepatitis B
• Hepatitis C
• Alcoholism
• Primary sclerosing cholangitis
• Choledochal cyst disease
• Polycystic liver disease
• Bile duct stones
• Clonorchiasis
• Caroli syndrome

A diagnosis of bile duct cancer can be upsetting and overwhelming. Educating yourself and finding support may help you face your cancer head on. Here are some of Everyday Health’s top resources for people with bile duct cancer:

The Cholangiocarcinoma Foundation

GI Cancers Alliance

Cholangiocarcinoma Support Group (Facebook)

American Cancer Society

Mayo Clinic

Cleveland Clinic

American Society of Clinical Oncology

Cancer Support Community