Bile duct cancer is rare, but it may be fatal. A person’s outlook will depend on the location of the cancer and the stage of the disease when they receive a diagnosis.

In this article, we discuss the different types of bile duct cancer, including the symptoms and causes. We also explore the risk factors and the stages of the disease, as well as its diagnosis, treatment, and prevention.

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Research estimates that 3,000–30,000 people in the United States have bile duct cancer, with about 8,000 people receiving a diagnosis every year.

Bile duct cancer, also known as cholangiocarcinoma (CCA), is cancer that affects the bile ducts.

The bile ducts are thin tubes that connect the liver to the small intestine. The primary role of the bile ducts is to act as a passage for bile from the liver and gallbladder into the small intestine. Bile aids in the digestion and absorption of fat in food.

Bile duct cancer occurs when old and abnormal cells in the bile duct start to grow without restrictions.

Bile duct cancer can occur in any part of the bile duct system. Experts classify bile duct cancers into three types depending on where they first appear:

  • Intrahepatic bile duct cancers start in the smaller bile duct branches inside the liver.
  • Perihilar or hilar bile duct cancers start at the hilum, which is where the left and right hepatic ducts join when leaving the liver.
  • Distal bile duct cancers occur further down the bile duct, closer to the small intestine.

People may refer to perihilar and distal bile duct cancers as extrahepatic bile duct cancer, as they start outside the liver. Cancers that start in different parts of the bile ducts can cause different symptoms.

The American Cancer Society (ACS) notes that bile duct cancers do not typically cause symptoms until a later stage in the disease. However, in some cases, the symptoms may become apparent sooner.

Symptoms that a person with bile duct cancer may experience include:

Although the exact cause of bile duct cancer is unknown, researchers believe that there is a link between bile duct cancer and factors that irritate and inflame the bile ducts. These factors may include:

Inflammation in the bile ducts may lead to DNA changes in bile duct cells, which may cause the uncontrolled growth of these cells.

Gene mutations may also increase the likelihood of bile duct cancer occurring. Some genes that may play a role in bile duct cancers include:

  • TP53
  • KRAS
  • HER2
  • ALK

Certain factors can increase the risk of a person developing bile duct cancer. Some risk factors include:

Medical professionals use the TNM staging system to stage bile duct cancer:

  • T refers to the tumor size and any spread of cancer into nearby tissue.
  • N refers to the spread of cancer to nearby lymph nodes.
  • M refers to metastasis, which means the spreading of cancer to other parts of the body.

All three types of bile duct cancers have slightly different staging systems. However, all three staging systems range from 0 to 4, with stage 4 being the most advanced type of cancer.

A doctor will likely begin the diagnostic process by taking a medical history from the person to determine whether they have risk factors for bile duct cancer. They will also ask the person about their symptoms.

The doctor may then perform a physical exam to look for signs of bile duct cancer.

For a more accurate diagnosis, the doctor will request blood tests and imaging tests to discover the type of bile cancer present.

Blood and function tests

A doctor may also order liver function tests. These tests measure how much bilirubin is in the blood. Bilirubin is the substance that causes jaundice. If there are problems in the bile duct, liver, or gallbladder, the level of bilirubin in the blood is likely to rise. Liver function tests will also check for:

Carcinoembryonic antigen (CEA) and CA 19-9 tumor marker tests look for an increase in the level of CEA and CA 19-9. Organs, tissues, and tumor cells release tumor markers into the blood, and an increase in the blood levels of these markers may indicate bile duct cancer.

Imaging tests

The doctor may also request imaging tests. These tests use X-rays, magnetic fields, or sound waves to produce a picture of the inside of a person’s body and visualize the bile ducts better. The resulting images may also help show whether cancer has spread to other body areas.

Imaging tests that a doctor may request include:

Biopsy samples

The doctor may also wish to take a sample of the suspected cancer so that a lab can examine it under a microscope and check for signs of cancer. This procedure is called a biopsy. The type of biopsies that a person may undergo are:

  • laparoscopy
  • percutaneous transhepatic cholangiography (PTC)
  • endoscopic retrograde cholangiopancreatography (ERCP)
  • endoscopic ultrasound (EUS)

The treatment for bile duct cancer may depend on whether the cancer is resectable or unresectable. Resectable bile duct cancers are those that doctors can remove completely with surgery. Unresectable bile duct cancers may be too difficult to remove completely.

The surgical options for bile duct cancer include:

  • Removal of the bile duct: A surgeon will surgically remove a part of the bile duct if the cancer is small and only affecting the bile duct.
  • Partial hepatectomy: This surgical procedure involves removing the cancerous part of the liver.
  • Whipple procedure: This procedure involves removing the head of the pancreas, the gallbladder, the stomach, part of the small intestine, and the bile duct.

A person may also undergo radiation therapy to kill the cancer cells and prevent them from growing. Radiation therapy involves using X-rays or a different type of radiation.

Chemotherapy is a common treatment approach that uses drugs to kill cells or stop them from dividing, preventing cancer cell growth.

Doctors use chemotherapy for unresectable bile duct cancers. The chemotherapy drug combinations that a person may take include:

  • gemcitabine and cisplatin
  • capecitabine and oxaliplatin
  • gemcitabine and oxaliplatin

A liver transplant may be a treatment option for people with perihilar bile duct cancer. This process involves removing the entire liver and replacing it with a healthy donor liver.

Researchers are investigating other therapies to treat bile duct cancer, including targeted drug therapy such as FGFR2 inhibitors and immunotherapy.

According to the ACS, there is no certain way to prevent bile duct cancer.

However, people can reduce their risk of developing bile duct cancer by:

  • maintaining a moderate weight
  • exercising regularly
  • following a balanced diet comprising plenty of fruits, vegetables, and whole grains
  • getting the vaccination for hepatitis B
  • avoiding or limiting alcohol consumption, if a person drinks
  • quitting smoking, if applicable

Below, we answer some common questions about bile duct cancer.

What is the survival rate for bile duct cancer?

According to the ACS, the overall 5-year relative survival rate for bile duct cancer is 9% for intrahepatic bile duct cancers and 10% for extrahepatic bile duct cancers.

Is bile duct cancer the same as liver cancer?

Although intrahepatic bile duct cancer occurs in the bile ducts within the liver, it is not liver cancer. Liver cancer is cancer that starts in the tissues of the liver itself. Cancer that starts in other cells and spreads to the liver is not primary liver cancer.

Is bile duct cancer aggressive?

Bile duct cancer is a rare but aggressive type of cancer. The 5-year relative survival rate for intrahepatic and extrahepatic bile duct cancers is either equal to or below 10%.

Where does bile duct cancer spread first?

Bile duct cancer is most likely to invade the surrounding tissue, such as nearby blood vessels, lymph nodes, and organs, such as the liver. It may then spread to other organs and bones.

Does chemotherapy work for bile duct cancer?

Chemotherapy may help treat bile duct cancer in some people. However, the data are unclear on the efficacy of chemotherapy in treating this cancer.

Survival rates for bile duct cancer largely depend on the location of the bile duct cancer and its stage.

The 5-year relative survival rate for localized bile duct cancers is 24% for localized intrahepatic bile duct cancers and 17% for localized extrahepatic bile duct cancers.

Early detection and effective treatment give people the best chance of a positive outcome.