Cholangiocarcinoma is a rare type of cancer that develops in the bile ducts that move digestive fluid from the liver to the small intestine.

The bile ducts are a network of tubes that connect the liver, gallbladder and small intestine. These ducts carry bile—a digestive fluid produced by the liver—to the gallbladder and small intestine.

Cholangiocarcinoma happens when cells in your bile ducts multiply out of control, forming a tumor. Bile duct cancer is most common in people older than 50.

Bile duct cancer does not usually show symptoms at an early stage. This means it is often diagnosed in its advanced stages, when it is more difficult to treat.

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Symptoms

Bile duct cancer symptoms can often be vague or resemble other medical conditions.

Some symptoms of bile duct cancer are caused by high levels of bilirubin, which is a yellow pigment produced when the body breaks down red blood cells. Bilirubin levels often increase in people with bile duct cancer because the cancer can block the flow of bilirubin from the liver, causing it to build up in the bloodstream.

Common cholangiocarcinoma symptoms include:

  • Jaundice: the yellowing of the skin and eyes due to the buildup of bilirubin
  • Itchy skin: persistent and unexplained itching that can be widespread
  • Dark urine and pale stools: noticeably darker urine, resulting from high levels of bilirubin and stools that appear unusually light or clay-colored
  • Abdominal pain: discomfort or pain in the upper right section of the abdomen
  • Unintended weight loss: significant weight loss without a change in diet or exercise
  • Fever: elevated body temperature, often without an apparent infection
  • Nausea and vomiting: feeling sickness and the presence of vomiting, leading to discomfort

If you experience any of these symptoms, it’s essential to see a health care provider for evaluation.

A multidisciplinary team of specialists at WashU Medicine manages bile duct cancer. This ensures a comprehensive and coordinated treatment plan.

Cholangiocarcinoma specialists at WashU Medicine


Causes and risk factors

Bile duct cancer is primarily caused by chronic inflammation of the bile ducts.

Several risk factors increase the likelihood of developing this cancer.

The exact cause of bile duct cancer remains unclear, but several risk factors can increase the likelihood of developing the disease:

  • Chronic bile duct inflammation: Also known as cholangitis, this can lead to narrowing of the ducts leading to serious complications.
  • Liver diseases such as hepatitis and cirrhosis: Chronic liver conditions can increase the risk of bile duct cancer.
  • Parasitic infections: Infections from liver flukes, while less common in North America, can elevate the risk.
  • Abnormalities in the bile ducts: Congenital or acquired abnormalities in the bile ducts can predispose people to cancer.
  • Age: The risk increases significantly with age, being most common in people over 50.
  • Family history of bile duct cancer: A family history of bile duct cancer can raise the likelihood of developing the disease.
  • Primary sclerosing cholangitis: This inflammatory condition of the bile ducts is a known risk factor.
  • Diabetes: Having diabetes may slightly increase the risk.
  • Exposure to certain chemicals: Long-term exposure to specific chemicals such as dioxins and nitrosamines can be a risk factor.

Screenings and diagnosis

The standard of care for bile duct cancer begins with a comprehensive diagnostic evaluation to confirm the presence and stage of the cancer. This typically involves:

  • Imaging rests: Ultrasound, CT scans, MRI scans, and cholangiography are used to visualize the bile ducts and identify tumors.
  • Biopsy: A sample of tissue may be obtained through endoscopic retrograde cholangiopancreatography (ERCP) or another procedure for pathological examination.
  • Blood tests: These can measure liver function and detect tumor markers specific to bile duct cancer.

A definitive diagnosis may require a biopsy, where a tissue sample is analyzed for cancer cells.

Getting ready for surgery

Taking steps to get healthy before surgery can help improve your outcomes and recovery.

WashU Medicine offers a “prehabilitation” program to help people get ready for surgery, including:

  • Physical activity
  • Lung health
  • Nutrition
  • Mindfulness

Procedures and what to expect

Treatment plans are highly individualized based on the stage of the disease and location of the cancer.

Resection (removal) of the cancer tissue and some surrounding healthy tissue is a likely treatment option.

Common procedures include:

  • Surgery: Surgical removal of the affected bile ducts and possibly surrounding tissues. In advanced cases, a liver transplant may be considered, if the disease is confined to the liver.
  • Radiation therapy: Types of radiation might Include external beam radiation (utilizes high-energy rays targeted at the tumor to kill cancer cells) and brachytherapy, which involves placing radioactive material inside or near the tumor.
  • Chemotherapy: Types of chemotherapy might include systemic chemotherapy, which uses drugs like gemcitabine and cisplatin are administered to target cancer cells throughout the body. Also localized chemotherapy, which is the direct injection of chemotherapy drugs into the bile ducts.
  • Biliary drainage: Procedures like stenting or percutaneous transhepatic cholangiography (PTC) may be used to alleviate symptoms caused by bile duct obstruction.

Exceptional cancer care


Recovery and rehabilitation

Regular follow-up appointments are crucial to monitor for recurrence and manage any long-term side effects.

Post-treatment care is vital for recovery. It typically includes:

  • Pain management: effective strategies to alleviate pain and discomfort associated with cancer and its treatment.
  • Nutritional support: tailored dietary plans to ensure adequate nutrition and manage potential malabsorption issues
  • Psychological counseling: mental health support to help patients cope with the emotional challenges of cancer diagnosis and treatment
  • Physical therapy: rehabilitation exercises to aid recovery, improve strength, and enhance mobility after surgical procedures

Managing medication

Your healthcare provider will guide you through managing medications post-treatment. This may include:

  • Pain management
  • Antibiotics to prevent infection
  • Medications to aid digestion