Bile Duct Cancer (Cholangiocarcinoma)

What is bile duct cancer (cholangiocarcinoma)?

One of the many functions of the liver is the production and transmittal of bile. Bile is the fluid necessary for the breakdown of food during the digestive process. The small tubes that conduct bile from the liver to the gallbladder to the intestines are “ducts”. Bile duct cancer (cholangiocarcinoma) is the formation of malignant (cancerous) tumors in these tubes or ducts.

Bile duct cancers are referred to by their locations in the system; proximally being inside the liver and distally being outside of the liver. There are two types of bile duct cancer, intrahepatic bile duct cancer, and extrahepatic bile duct cancer.

1. Intrahepatic bile duct cancer is the condition where the cancer cells form on the bile ducts inside the liver.
2. Extrahepatic bile duct cancer forms in the bile ducts outside the liver and is the most common form of this disease. There are two forms of extrahepatic bile duct cancer: perihilar cholangiocarcinoma and distal cholangiocarcinoma.
   1. Perihilar cholangiocarcinoma is centered in the area where the right and left bile ducts exit the liver to form a common bile duct. This area is known as the hilum region.
   2. Distal cholangiocarcinoma is located in the distal region. This is the area where the common bile duct passes through the pancreas and enters the small intestines.

What are some of the risk factors?

A risk factor is anything that can lead to disease. Having risk factors does not mean you will develop the disease. Likewise, not having any of the known risk factors does not mean you cannot develop the disease. Most biliary cancers occur without any specific risk. However, known risk factors can include:

• Primary Sclerosing Cholangitis (PSC) which causes the blockage of the bile ducts because of scarring or inflammation
• Bile duct cysts which block the passage of bile and cause swelling, inflammation, and infections
• Chronic ulcerative colitis which occurs when the large intestines are damaged by sores, ulcers, and inflammation
• Germline mutations, which are inherited genetic changes.

It is important to consult your physician if you have a specific risk factor, or a family history of these risks.

What are the symptoms?

Symptoms can include:

• Jaundice (yellowing of the skin or whites of the eyes)
• Dark urine
• Clay colored stool
• Pain in the abdomen
• Fever
• Pruritus (itchy skin)
• Nausea and vomiting
• Weight loss for an unknown reason
• Chills
• Loss of appetite

How is it diagnosed?

Tests and procedures to detect, diagnose, and stage bile duct cancer are usually done at the same time. Staging is the process used to find out if cancer cells have spread within and around the bile ducts to lymph nodes, or distant parts of the body.

The following tests and procedures may be used:

• Physical exam and health history
  An overall examination of the body is performed to check general signs of health. The individual’s health history and that of family members can determine if there is any predisposition to biliary cancer.
• Liver function tests
  Bilirubin, AST, and ALT levels are measured. Elevated amounts of these enzymes can be a sign of liver disease that may be caused by bile duct cancer.
• Laboratory tests
  These medical procedures test samples of tissue, blood, urine, or other substances in the body and help diagnose disease, plan and assess treatment, and monitor the disease.
• Carcinoembryonic antigen (CEA) and CA 19-9 tumor marker test
  Tumor marker tests are procedures in which a sample of blood, urine, or tissue is evaluated for the presence of substances made either cancer cells or by normal cells in response to cancer. Higher than normal levels of CEA and CA 19-9 may be related to cholangiocarcinoma but testing for these tumor markers is not diagnostic.
• Imaging Tests
  Ultrasound exam, CT scan (CAT scan), MRI (magnetic resonance imaging), MRCP (magnetic resonance cholangiopancreatography) are high-tech, non-invasive procedures that can aid in diagnosis by showing signs of liver damage or disease.
• Biopsy
  During a biopsy procedure, cells and tissues are removed and identified under a microscope by a pathologist to check for signs of cancer. This is a mildly invasive procedure using a needle that is guided by information obtained from the imaging tests. The type of biopsy used will depend on the information being sought. A critical part of the biopsy will help assess genetic bile duct cancer-related changes in the tumor. These mutations may be targets for therapy and thus a more personalized treatment plan.

How is it treated?

There are certain factors to be considered when discussing treatment options. These include the location of the cancer, whether it has spread to other organs, the possibility of completely removing the cancer with surgery, and other health challenges.

• Surgery
  Whenever possible, surgery is the main treatment for bile duct cancers. The goal is to completely remove the cancer from the body.
• Systemic therapy
  Systemic therapy is cancer treatment that targets the entire body. Chemotherapy, targeted therapy, immunotherapy, and therapies being studied in clinical trials are types of systemic therapies.
  • When systemic therapy is given in addition to surgery it is called adjuvant therapy. The aim is to increase the effectiveness of the surgery and prevent any recurrence of the cancer. When given this type of therapy is given before surgery it is called neoadjuvant. When given before and after it is called periadjuvant.
  • In cases in which surgery is not feasible because of risk factors, or because the cancer has spread to other sites, then a form of systemic therapy would be recommended.
• Liver Transplant
  Complete removal of the liver and bile ducts followed by a liver transplant may be an option for certain individuals.

Learn how the Liver Cancer Program at the National Institutes of Health, National Cancer Institute's Center for Cancer Research can help all liver cancer patients. This video features Tim Greten, MD, Co-Director of the NCI CCR Liver Cancer Program who provides information on clinical trials, liver cancer treatments and advice for patients.

Are clinical trials available?

Yes, there are clinical trials available for those with cholangiocarcinoma.

Clinical trials are research studies that test how well new medical approaches work in people. Before an experimental treatment for cholangiocarcinoma can be tested on human subjects in a clinical trial, it first must have shown benefit in laboratory testing or animal research studies. Only the most promising treatments are then moved into clinical trials, with the goal of identifying new ways to safely and effectively prevent, screen for, diagnose, or treat a disease.

The following websites include information about clinical trials. Always consult with your physician before signing up for a clinical trial:

• The ABC's of Clinical Trials
• https://www.antidote.me/
• https://www.nih.gov/health-information/nih-clinical-research-trials-you
• https://clinicaltrials.gov/
• https://cholangiocarcinoma.org/professionals/research/clinical-trials/

What questions should I ask my doctor?

• Is the cancer only in my bile ducts?
• Do I need further testing before we consider treatment options?
• Will genetic testing be beneficial?
• Are you experienced in treating this type of cancer?
• Do I need to see any other kinds of doctors?
• How much experience do you have treating this type of cancer?
• Can surgery remove the cancer?
• What is the goal of treatment?
• What side effects are common with the treatment you are recommending? How long are they likely to last?
• How quickly do we need to decide on treatment?
• How long will treatment last? What will it be like? Where will it be done?
• How will treatment affect my daily activities?
• What are the chances my cancer can be cured with these treatment plans?
• What would my options be if the treatment doesn’t work or if the cancer comes back?
• What type of follow up will be needed?
• Will genetic testing be beneficial to my treatment plan?

Register for an on-demand webinar

Watch a panel of liver cancer specialists from Memorial Sloan Kettering in a 1-hour on-demand webinar on new and emerging treatments for liver cancer.  You will gain a better understanding of how those therapies work and which patients are most likely to benefit from which therapies.  Learn about strategies to help with treatment-associated side effects. Learn how to be an advocate for better care and use support resources.

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Related Information and Resources

• https://cholangiocarcinoma.org/
• https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq
• https://rarediseases.org/rare-diseases/cholangiocarcinoma/

Funding generously provided by Servier Pharmaceuticals, with additional support from QED Therapeutics, Inc. and Zymeworks, Inc.

Sources of Information

• Cholangiocarcinoma Foundation
• National Cancer Institute

Patient Stories

Last updated on January 18th, 2024 at 02:48 pm