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Bile Duct Cancer (Cholangiocarcinoma)

Contact the UPMC Center for Liver Diseases

To make an appointment with a hepatologist at the UPMC Center for Liver Diseases, call 412-647-1170 or fill out our contact form.


What Is Bile Duct Cancer (Cholangiocarcinoma)?

Bile duct cancer is cancer in the tubes that carry bile from the liver to the gallbladder. Bile is fluid your liver makes and uses to digest fats in the food you eat.

Several different tubes form your liver's bile duct network. This network includes small ducts that join to form the left and right hepatic ducts inside the liver.

These ducts then merge and become the common hepatic duct outside the liver.

The common hepatic duct ends at the cystic duct, where the two ducts merge to form the common bile duct.

Bile duct cancer can occur in any of these tubes.

Bile duct cancer isn't very common. Doctors only diagnose about 8,000 people in the U.S. each year.

Types of bile duct cancer

Bile duct cancer can begin in any of the bile ducts inside or outside the liver.

These cancers have distinct names, based on the cancer's location.

Extrahepatic cholangiocarcinoma starts in the bile ducts outside the liver, including the common hepatic duct and the cystic duct.

There are two types of extrahepatic bile duct cancers:

  • Perihilar cholangiocarcinoma, or Klatskin tumor. This cancer forms in the hilum, the place where bile ducts merge when leaving the liver. This is the most common type of bile duct cancer.
  • Distal cholangiocarcinoma. This cancer forms in bile ducts outside the liver.

Intrahepatic cholangiocarcinoma is cancer that grows in the bile ducts inside the liver. This type of bile duct cancer is less common than extrahepatic cancers.

Bile duct cancer causes

Cancer occurs when cells grow in ways that aren't normal. Changes in certain genes cause this abnormal growth.

Researchers don't think parents pass these gene changes to their children, but they're still studying this.

Some people with bile duct cancer form mutations in the genes that control how cells divide, causing tumors to grow. Others have oncogenes, or gene changes, that can cause cells to become cancerous.

Bile duct cancer risk factors and complications

You may be at higher risk for bile duct cancer if you have:

  • Chronic ulcerative colitis, or ulcers (sores) in the lining of the colon or rectum.
  • Primary sclerosing cholangitis, or scarred or inflamed bile ducts.
  • Bile duct cysts that cause swelling or infection.
  • Hepatitis B or C.
  • Cirrhosis of the liver.
  • A history of working with certain manufacturing chemicals.

Bile duct cancer can cause liver problems such as cirrhosis and liver failure.

Sometimes, cancer can spread to other parts of the body.

Why choose the Center for Liver Diseases for HCC care?

Our expert liver doctors use the latest, most advanced treatments for bile duct cancer.

And we partner with the UPMC Liver Cancer Center and the Liver Transplant Program to tailor treatment plans to meet your needs.

Bile Duct Cancer Symptoms and Diagnosis

Sometimes, people with bile duct cancer don't have any symptoms when it first begins, known as early-stage cancer.

If cancer spreads or tumors block the bile ducts, symptoms can include:

  • Stomach pain.
  • Fever.
  • Jaundice (a yellow color of the skin and eyes).
  • Itchy skin.
  • Dark urine.
  • Light, or clay-colored, stools.
  • Unexplained weight loss.
  • Nausea.
  • Fatigue.

Diagnosing bile duct cancer

Doctors will start with a physical exam to diagnose bile duct cancer.

They also use imaging tests to look for liver damage or signs that your liver isn't working as it should.

These tests include:

  • CT scans.
  • MRI.
  • Ultrasound.

Magnetic resonance cholangiopancreatography (MRCP) is a specialized imaging technique. It takes detailed pictures of the inside of the liver and bile ducts. Radio waves and magnets create pictures that doctors can see on a computer.

Doctors also use blood tests to help diagnose bile duct cancer, including:

  • Liver function tests: Look for elevated levels of bilirubin, a part of bile made when red blood cells break down. They also look for alkaline phosphatase, a liver enzyme. High levels of these substances might be a sign of bile duct cancer.
  • Tumor marker tests: Look for substances called tumor markers, which the body makes when cancer is growing.

Doctors may also confirm bile duct cancer with a biopsy, or tissue sample.

Bile Duct Cancer Treatment

Doctors will look at your type of bile duct cancer and your overall health when designing your treatment.

Lifestyle changes to treat bile duct cancer

Lifestyle changes can't cure your cancer, but they can help keep your liver healthy.

These include:

  • Staying at a healthy weight.
  • Exercising.
  • Not smoking.
  • Avoiding heavy drinking.

Medical treatments for bile duct cancer

Doctors have a few options for treating bile duct cancer, including:

  • Chemo: Drugs that kill cancer cells by stopping growth during the cell's lifecycle. Chemo affects both healthy cells and cancer cells.
  • Immunotherapy: Drugs that help the immune system fight cancer.
  • Targeted therapy: Drugs that attack specific types of cancer cells, while leaving healthy cells alone.
  • Radioembolization: Radioactive beads deliver radiation to tumors to shrink them. Doctors insert these beads into a thin tube, or catheter, in an artery in your thigh. Then they feed the tube into the hepatic artery to prevent blood flow to the tumor. Sometimes, they add chemo.

Surgery to treat bile duct cancer

Doctors use different surgeries to treat bile duct cancer, such as:

  • Bile duct removal: To remove the bile duct if the cancer hasn't spread.
  • Partial hepatectomy: To remove the tumor and some of the surrounding tissue, leaving healthy tissue intact.
  • Liver transplant: To remove the diseased liver and replace it with a healthy liver from a living donor.