Bile Duct Cancer

Bile duct cancer is an uncommon type of cancer that starts in the bile ducts. These ducts are part of your digestive system. Bile is a fluid made by the liver that helps in digestion. Bile ducts carry bile from your liver and gallbladder to the small intestine. 

The management of bile duct cancer can be complicated, so it’s important to be evaluated early and get specialized care.

What is bile duct cancer?

Bile duct cancer is a type of cancer that happens when abnormal cells form in the bile ducts and grow out of control.

You may see this cancer called cholangiocarcinoma or biliary tract cancer (BTC). There are several subtypes of bile duct cancer, based on where the cancer starts in the bile duct system.

Bile duct cancer is found most often in older adults. It can grow quietly for a long time before it causes symptoms.

Where bile duct cancer develops

Bile duct cancer can develop in different parts of the bile duct system. Symptoms, treatment and outcomes are different based on where the cancer is located.

Main types of bile duct cancer include:

• Intrahepatic cholangiocarcinoma (ICC): This type develops in the bile ducts within the substance of the liver. While a type of primary liver cancer, they are different from the more common kind of liver cancer, hepatocellular carcinoma (HCC). 
• Perihilar cholangiocarcinoma: These types form where the bile ducts exit from the liver, in an area called the common hepatic duct. They are also called Klatskin tumors.  Perihilar or hilar cancers are more likely than other types to block bile ducts and cause jaundice, so they are sometimes detected sooner. 
• Extrahepatic bile duct cancer: These types originate in the bile duct outside of the liver, including in the bile duct within the pancreas (distal cholangiocarcinoma). They also block the bile flow and often present with jaundice.

How bile duct cancer differs from liver and gallbladder cancer

Gallbladder cancer is also of bile duct origin, although somewhat different from the other cholangiocarcinoma types. These cancers can have symptoms similar to gallstone disease or even sometimes found incidentally when the gallbladder is removed for other reasons.

Even though bile duct cancer is closely related to liver and gallbladder cancers, these cancers all have different cell types, risk factors and treatment approaches. 

Bile duct cancer may spread along the bile ducts and into lymph nodes, which can make surgery and staging more difficult than it is for some liver or gallbladder cancers.

Learn more

• Liver cancer
• Gallbladder cancer

Symptoms of bile duct cancer

Early bile duct cancer may not cause symptoms. As the cancer grows, symptoms may be different depending on where the cancer is in the bile ducts. They may include:

• Yellowing of the skin or eyes (jaundice)
• Dark urine
• Greasy, pale or colored stools
• Itchy skin
• Pain in the upper right side of the abdomen, middle of the abdomen or back
• Unexplained weight loss
• Nausea and vomiting
• Loss of appetite
• Fatigue
• Weakness
• Fever
• Night sweats
• Not feeling well

These symptoms can overlap with other liver and digestive diseases. If you have them, you should contact a health care provider for guidance.

Sometimes bile duct cancer is diagnosed without any symptoms. They can be found incidentally when a scan or ultrasound is done for some other unrelated reason or detected by abnormal liver blood tests. 

Risk factors for bile duct cancer

Conditions that increase your risk include:

• Primary sclerosing cholangitis, which is a chronic (long-lasting) bile duct disease
• Long-term inflammation or scarring of the bile ducts
• Certain inherited bile duct conditions
• Bile duct cysts or stones
• Structural issues where the bile duct joins the pancreatic duct
• Chronic liver diseases
• Diabetes
• Obesity
• Tobacco use
• Exposure to certain toxins
• Infection with a liver fluke, found in some parts of Southeast Asia

While these risk factors are associated with higher incidence, most patients who develop bile duct cancer have no risk factors. 

How bile duct cancer is diagnosed

Testing may include:

• Imaging studies like ultrasound, MRI and CT scans to look at the bile ducts and nearby organs
• Blood tests to look for tumor markers and check how well the liver is functioning
• Specialized procedures such as ERCP (endoscopic retrograde cholangiopancreatography) to look at the bile ducts, clear blockages and take tissue samples
• A biopsy to confirm cancer, if needed
• In some cases, PET scans or endoscopic ultrasound (EUS) to see if the cancer has spread

It’s important to have an accurate diagnosis of bile duct cancer, because that helps health care providers make the right treatment decisions. 

Bile duct cancer may metastasize (spread) to nearby lymph nodes, the liver and other organs if it’s not found early.

Treatment options for bile duct cancer

Treatment depends on the cancer’s location and stage and your overall health.

A personalized care plan may include:

• Surgery to remove the tumor when possible. That may include removing part of the bile duct, part of the liver, nearby lymph nodes and sometimes the gallbladder, pancreas or small intestine. Surgery offers the best chance for a cure. 
• Chemotherapy, before or after surgery or when the cancer has spread or cannot be removed.
• Radiation therapy, externally or directly to the tumor. Radiation is occasionally used and may help shrink tumors, manage symptoms or treat cancer that remains after surgery.
• Liver transplant, in some cases.
• Targeted treatments for cancers with certain genetic changes.
• Immunotherapy, which helps the immune system fight cancer.
• Supportive treatments like procedures to open blocked bile ducts, pain management, symptom relief and nutrition support.
• Other liver directed therapies. In selected cases, one might consider other therapies directed to the liver. These may include intraarterial therapies (TACE or TARE), liver chemotherapy pump (HAI), or histotripsy.

Because many bile duct cancers are not found until they are advanced, care may focus on controlling the cancer, relieving symptoms and helping you live well for as long as possible.

Why specialized care matters

Bile duct cancer can be difficult to diagnose and treat. Because it’s rare, it’s best to get treatment by a multidisciplinary team that has experience in these types of cancers.

Specialized care helps make sure:

• The type of cancer is diagnosed accurately
• Treatment is planned appropriately
• Care is coordinated across surgery, oncology and imaging
• Complex needs and side effects are managed properly
• You’re connected with emerging therapies and clinical trials

Early referral to an experienced care team can improve your outcome and your quality of life.

Why choose Banner MD Anderson for bile duct cancer care

At Banner MD Anderson Cancer Center, we provide comprehensive care for people with bile duct cancer, including advanced imaging, expert diagnosis and coordinated treatment planning by a highly skilled and experienced team. 

Your care team may include hepatobiliary surgeons, medical oncologists, radiation oncologists, interventional radiologists, gastroenterologists, radiologists, pathologists and care specialists.

Our cancer specialists work together to create personal care plans and guide you through every step of your care. 

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