The information on this page was reviewed and approved by
Maurie Markman, MD, President, Medicine & Science at CTCA.

This page was updated on September 21, 2021.

About bile duct cancer

Bile duct cancer is a rare disease that begins in the bile ducts, which are thin tubes that transport digestive fluid (called bile) from the liver and gallbladder into the small intestine.

About 8,000 people in the United States are diagnosed with bile duct cancer annually, according to the American Cancer Society. When bile duct tumors block the flow of bile and bilirubin from the liver, a person with this cancer may exhibit jaundice, a yellowing of the skin and the whites of the eyes. The disease may also cause symptoms such as itching, abdominal pain and weight loss. Due to the location of the bile ducts deep inside the body, these tumors are rarely caught early. Surgery is typically the first line of treatment.

What can cause cancer of the bile duct?

The bile ducts play a significant role in the proper functioning of the digestive system. Problems with the bile duct system may cause digestive issues or jaundice, or they may develop into a chronic disease.

Risk factors for bile duct cancer vary but may include:

  • Exposure to hazardous chemicals, such as dioxins, nitrosamines, polychlorinated biphenyls (PCBs), asbestos, radon or Thorotrast, a radiologic contrast agent used until the mid-1950s
  • Chronic irritation or inflammation of the bile duct, possibly brought on by bile duct stones (similar to gallstones), ulcerative colitis or primary sclerosing cholangitis (PSC)
  • Liver or bile duct diseases, such as polycystic liver disease, pancreatitis, irritable bowel syndrome, choledochal cysts or Caroli’s syndrome
  • Parasitic infections, brought on by a water-borne parasite called liver fluke

learn more about risk factors for bile duct cancer

Who gets bile duct cancer?

More than 60 percent of bile duct cancer patients are 65 years or older, and most cases do not appear to have a link to family history.

Smoking and obesity may result in an increased risk of bile duct cancer, as may excessive alcohol use. Alcohol use can also cause cirrhosis of the liver and increase a person’s liver cancer risks.

Bile duct cancer types

Cancer can occur in any part of the bile duct. The location of the primary tumor (inside or outside the liver) and the kind of cancer cells involved determine the types of bile duct cancer.

Most bile duct cancers develop in the ducts outside the liver. These are called extrahepatic bile duct cancers and include:

  • Bile duct adenocarcinomas, also called cholangiocarcinoma, which form in the cells of the mucous gland lining the inside of the bile duct
  • Hilar (or perihilar) bile duct cancers, also called Klatskin tumors, which form in the hilum region where hepatic duct branches leave the liver
  • Distal bile duct cancers, which occur near the small intestine

Intrahepatic bile duct cancer begins in the smaller duct branches of the liver. They are sometimes misdiagnosed as liver cancer, though both are typically treated the same way.

Learn more about bile duct cancer types

Bile duct cancer symptoms

Bile duct cancer symptoms typically do not appear in the early stages of the disease. As the disease progresses, cancerous cells spread first to the outer lining of the liver and then to structures near the liver, such as the duodenum, common bile duct, colon, stomach, diaphragm, etc. The bile ducts are located deep inside the body, so early tumors aren’t easily detected during routine physical exams.

The earliest and most common symptoms of bile duct cancer are related to abnormal liver functions. These may also include jaundice, loss of appetite, weight loss or changes in stool or urine (light colored stools and dark urine).

Other bile duct cancer (cholangiocarcinoma) symptoms may include:

  • Itchy skin
  • Enlarged abdominal mass or bloated feeling
  • Abdominal pain
  • Nausea
  • Fever

Learn more about symptoms of bile duct cancer

Diagnosing bile duct cancer

At CTCA®, a variety of tests and tools is used for diagnosing bile duct cancer, evaluating the disease and customizing a treatment plan for each patient. Throughout treatment, imaging and laboratory tests track the size of the tumors, monitor the response to treatment and help facilitate treatment plan modifications when needed.

The following procedures are commonly used for diagnosing bile duct cancer:

  • Laboratory tests, including liver function tests (LFTs) and blood tests for tumor markers
  • Endoscopic or laparoscopic ultrasound
  • Computed tomography scan (CT scan)
  • Magnetic resonance imaging (MRI) scan
  • Cholangiography (dye injection and X-rays), including endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTC) and magnetic resonance cholangiopancreatography (MRCP)

Learn more about diagnostic procedures for bile duct cancer

Bile duct cancer treatments

Treatment options for bile duct cancer include a variety of techniques in the areas of surgery, radiation and gastroenterology. These options may include:

  • Surgery for bile duct cancers that can be completely removed (resected), such as a partial hepatectomy (partial liver removal) or removal of the bile duct, nearby lymph nodes and part of the liver, gallbladder, pancreas and/or small intestine
  • Surgery for cancers that cannot be removed (unresectable), such as insertion of a biliary stent or biliary catheter to keep the bile duct open, or a biliary bypass to allow bile to reach the small intestine
  • ERCP, to allow removal of biopsy samples, to provide relief from a bile duct obstruction or to insert a stent
  • Balloon dilation, to remove obstruction
  • Photodynamic therapy (PDT)
  • Radiation therapy, including external beam radiation therapy (EBRT), intensity modulated radiation therapy (IMRT), intraoperative radiation therapy (IORT) and TomoTherapy®

Learn more about treatment options for bile duct cancer