Bile Duct Cancer

Bile duct cancers can be categorized into three types depending on their location in the body: intrahepatic, perihilar and distal bile.

Intrahepatic bile duct cancer

Intrahepatic bile duct cancers start inside the bile ducts in the liver. Intrahepatic means “inside the liver.”

Perihilar bile duct cancer

Perihilar (also called hilar) bile duct cancers start where the bile ducts exit the liver. These are considered extrahepatic, or “outside the liver.” They also are known as Klatskin tumors.

Distal bile duct cancer

Distal bile duct cancers are extrahepatic cancers that begin closer to the small intestine.

Adenocarcinomas include most bile duct cancers and start in the mucous gland cells that line the bile duct.

Bile duct cancers also may be categorized by how the cancer cells look under a microscope, as with adenocarcinomas. Much less common types of bile duct cancers include sarcomas, lymphomas and small cell cancers.

What causes bile duct cancer is unknown, but several risk factors do seem to increase the likelihood of developing it. The most common risk factors for bile duct cancer include chronic diseases of the liver or bile ducts. Ongoing liver or bile duct disease can cause inflammation, which is linked to cancer.

Conditions that may raise the risk of bile duct cancer include:

Primary sclerosing cholangitis

This inflammation of the bile ducts forms scar tissue.

Bile duct stones

These small, hard masses form in the bile duct.

Choledochal cysts

These bile-filled sacs connect to the bile ducts.

Cirrhosis

Damage to the liver from irritants (such as alcohol) and diseases (such as hepatitis) can raise the risk.

Hepatitis

Long-term infections with hepatitis B virus or hepatitis C virus can lead to cirrhosis, which raises the risk.

Additional risk factors include:

Reflux

People who have a bile duct defect that allows digestive juices from the pancreas to flow back (reflux) into the bile ducts have a higher risk of bile duct cancer.

Inflammatory bowel disease

Inflammatory bowel diseases, including ulcerative colitis and Crohn’s disease, increase the risk.

Age

Bile duct cancer is most often diagnosed after age 60.

Weight

Excess weight may increase the risk of gallstone and bile duct stones, which in turn increase the risk of these cancers.

Exposure to Thorotrast

A radioactive substance called Thorotrast (thorium dioxide), used in X-rays until the 1950s, increased the risk for bile duct cancer.

Alcohol

Alcohol use may increase the risk of intrahepatic (inside the liver) bile duct cancer, especially in people who have liver problems caused by alcohol use.

Family history

A family history of bile duct cancer may raise the risk, but most people who have bile duct cancer don’t have a family history of the disease. Inherited gene mutations don’t seem to be a risk factor.

If you’re concerned about your risk for bile duct cancer, talk to your doctor 

For some types of cancer, routine screening exams can help detect tumors early, even if an individual does not have symptoms or a high risk of developing the disease. But there’s no effective bile duct cancer screening exam, because no tests can detect it at an early stage. Also, because bile ducts are deep inside the body, small tumors cannot be found during routine physical exams. Most bile duct cancers aren’t found until they have begun to cause symptoms.

Bile duct cancer cannot be completely prevented, but you may be able to reduce your risk by taking these steps:

• Maintain a healthy weight
• Get a hepatitis B virus (HBV) vaccine to prevent infection
• Avoid hepatitis B virus (HBV) and hepatitis C virus, and get treatment if you are infected
• Limit alcohol use

Bile duct cancer symptoms are rare in the early stages of the disease. But if cancer cells block a bile duct early in the disease, symptoms may appear sooner.

Symptoms of bile duct cancer may include:

• Jaundice, or yellowing of the skin and eyes
• Itchy skin
• Dark urine
• Light-colored or greasy stools
• Pain in the abdomen
• Unintended weight loss
• Loss of appetite
• Fever
• Nausea or vomiting

Having any of these symptoms doesn’t mean you have bile duct cancer. In fact, many of these symptoms are likely to be caused by other conditions. For example, gallstones may cause many of these symptoms. Even so, if you have symptoms that persist or worsen, it’s important to have them checked by a doctor so that the cause can be found and treated, if needed.

Scripps cancer specialists are experts in diagnosing and staging bile duct cancer to plan the best course of treatment. Our gastroenterologists, pathologists and radiologists use advanced diagnostic testing methods to detect bile duct cancer.

Blood tests

Blood tests check for substances called tumor markers, bilirubin and other indicators of cancer in the blood.

Ultrasound

Ultrasound uses sound waves to show any growths on the liver, which may be tumors.

Computed tomography (CT) scan

A CT scan uses X-rays to provide detail about the size, shape and location of any growths in the liver or surrounding areas.

Magnetic resonance imaging (MRI)

An MRI uses radio waves to provide detailed images of the liver and any abnormal growths.

Biopsy

A gastroenterologist usually performs a biopsy of the suspicious tissue through an upper endoscopy. This procedure involves the insertion of a thin tube with a light and camera at the end to explore the upper digestive tract. Recovery is usually very quick after this procedure. Following the biopsy, a pathologist will analyze the tissue sample and test it for any abnormalities.

Doctors determine a patient’s bile duct cancer stage by how far the cancer has spread from where it originally started. Each type of bile duct cancer has its own staging system.

Intrahepatic bile duct cancer

Stage 0: Abnormal cells are found in the innermost layer of tissue lining the intrahepatic bile duct (the bile duct inside the liver). These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.

Stage I: There’s one tumor that has spread into the intrahepatic bile duct, but it has not spread into any blood vessels.

Stage II: There’s one tumor that has spread through the wall of the bile duct and into a blood vessel, or there are multiple tumors that may have spread into a blood vessel.

Stage III: The tumor has spread through the tissue that lines the abdominal wall, or it has spread to organs or tissues near the liver, such as the duodenum, colon and stomach.

Stage IVA: The cancer has spread along the outside of the intrahepatic bile ducts, or the cancer has spread to nearby lymph nodes.

Stage IVB: The cancer has spread to organs in other parts of the body.

Perihilar bile duct cancer

Stage 0: Abnormal cells are found in the innermost layer of tissue lining the perihilar bile duct (where the bile ducts exit the liver). These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.

Stage I: Cancer has formed in the innermost layer of the wall of the perihilar bile duct and has spread into the muscle layer or fibrous tissue layer of the wall.

Stage II: Cancer has spread through the wall of the perihilar bile duct to nearby fatty tissue or to the liver.

Stage IIIA: Cancer has spread to branches on one side of the hepatic artery that supplies blood to the liver and pancreas, or of the portal vein that moves blood from the spleen and intestines to the liver.

Stage IIIB: Cancer has spread to nearby lymph nodes. Cancer may have spread into the wall of the perihilar bile duct, or through the wall to nearby fatty tissue and liver, or to branches on one side of the hepatic artery or of the portal vein.

Stage IVA: Cancer has spread to one or more of the following:

• The main part of the portal vein and/or common hepatic artery
• The branches of the portal vein and/or common hepatic artery on both sides
• The right hepatic duct and the left branch of the hepatic artery or of the portal vein
• The left hepatic duct and the right branch of the hepatic artery or of the portal vein

Stage IVB: Cancer has spread to lymph nodes in more distant parts of the abdomen, or to organs in other parts of the body.

Distal extrahepatic bile duct cancer

Stage 0: Abnormal cells are found in the innermost layer of tissue lining the distal extrahepatic bile duct (the bile ducts closer to the small intestine). These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.

Stage IA: Cancer has formed and is found in the distal extrahepatic bile duct wall only.

Stage IB: Cancer has formed and has spread through the wall of the distal extrahepatic bile duct but has not spread to nearby organs.

Stage IIA: Cancer has spread from the distal extrahepatic bile duct to the gallbladder, pancreas, duodenum or other nearby organs.

Stage IIB: Cancer has spread from the distal extrahepatic bile duct to nearby lymph nodes. Cancer may have spread through the wall of the duct or to nearby organs.

Stage III: Cancer has spread to the large vessels that carry blood to the organs in the abdomen. Cancer may have spread to nearby lymph nodes.

Stage IV: Cancer has spread to organs in distant parts of the body.

Resectable and unresectable bile duct cancer

Treatment also depends on whether the cancer is resectable or unresectable.

• Resectable bile duct cancer is when the cancer can be removed completely by surgery.
• Unresectable bile duct cancer is when the cancer cannot be removed completely by surgery.

No one wants to hear that they have cancer. You may feel frightened, overwhelmed, anxious or sad. All of these feelings are perfectly normal. The more you learn about your diagnosis and next steps, the sooner you can begin to make informed decisions about your treatment.

Questions and considerations

Here are some questions you may want to ask your doctor or health insurance provider regarding your bile duct cancer diagnosis:

• Should I get a second opinion?
• How do I find a specialist?
• What is my cancer treatment plan?
• Will I have to miss work/school?
• What are the side effects of bile duct cancer treatment?
• How successful is my treatment likely to be?
• What costs will be covered by insurance?
• Which costs will I be responsible for?

Your Scripps cancer care team is here to help you find the answers you need to take an active role in your care. In addition, there are a number of community resources that provide education, information and helpful resources — often at no cost.

Learn more about the many cancer patient resources available to you and your loved ones at Scripps.

Our bile duct cancer specialists take a collaborative approach to your care, routinely meeting to review each patient’s unique needs and precisely diagnose and stage the cancer in order to develop a personalized treatment strategy. See how you're the center of care at Scripps.

Surgery is the best treatment for early-stage bile duct cancer, but many cases are not detected early enough for surgery. Radiation therapy may be used before surgery to shrink the tumor, after surgery to help prevent cancer from returning, or instead of surgery if the cancer cannot be completely removed. Chemotherapy also may be used with radiation.

When cancer has spread too far to be completely removed, Scripps surgeons may perform palliative surgery to help relieve or prevent symptoms.

Radiation therapy

Scripps has the most comprehensive radiation therapy offerings in San Diego for bile duct cancer, with advanced technology that precisely targets tumors.

3-D conformal radiation therapy

Our radiation oncologists can visualize a patient’s anatomy in 3-D, enabling them to match the radiation dose to the shape of the tumor while minimizing radiation exposure to surrounding healthy tissues and organs.

Intensity-modulated radiation therapy (IMRT)

Using this advanced form of radiation therapy, our radiation oncologists can specify the dose of radiation for the tumor while restricting the dose to healthy tissues.

Chemotherapy

Chemotherapy may be used as part of a treatment plan with the aim of eliminating cancer cells or lowering the risks that the cancer may return.

Some bile duct cancer treatment plans may involve clinical trials. Talk with your physician about whether a clinical trial is right for you. See a list of clinical trials that are currently enrolling patients.

Your Scripps cancer team will develop a customized treatment plan outlining the treatments and therapies we recommend for your care. Before you begin treatment, you and your physician will review your plan together and discuss any questions or concerns you may have.

Your bile duct cancer treatment plan generally includes:

• Family and medical history
• A summary of your cancer diagnosis and staging information
• Diagnostic testing completed (e.g., imaging, biopsy, lab tests)
• Plans for surgery, radiation, chemotherapy and/or other treatments
• Potential side effects of treatments
• Contact information for your cancer care team members

Along with helping you understand your course of treatment and encouraging discussion between you and your physician, your plan helps your team coordinate your care. You also can share your treatment plan with other providers who may be involved in your medical care.

Your bile duct cancer care team includes health and medical professionals from a wide range of specialties. Scripps Cancer Center will customize your team to ensure you have the expertise and support you need. 

Your team may include:

• Gastroenterologists
• Surgeons and surgical oncologists
• Radiation oncologists
• Medical oncologists
• Registered nurses
• Nurse navigators
• Dietitians
• Oncology social workers

Visit your cancer care team for more information on Scripps Cancer Center's multi-disciplinary approach to treatment.

In support of our patients, survivors, their family members and the community, we host a handful of cancer support groups as well as a range of free workshops and health and wellness events on a number of topics such as:

• Nutrition
• Expressive writing
• Yoga
• Meditation

Check the current list of support offerings or contact your oncology social worker or cancer care coordinator.

For info about other cancer support groups in the San Diego community, call the American Cancer Society at 800-227-2345.

We are here for you — not only as your oncologists, but as a robust multidisciplinary team of experts who understands that your cancer journey is about much more than your medical treatment. Specifically, Scripps Cancer Center offers a variety of patient support services to ensure your physical, psychological and emotional well-being as well as resources for dealing with the logistical and financial aspects of cancer care. Our services and resources include:

• Oncology nurses and nurse navigators with extensive clinical expertise in cancer care to help guide you and your caregivers to make informed decisions and ensure your optimal care.
• Palliative care to provide an extra layer of supportive care to manage pain and relieve symptoms, offer emotional and spiritual support, and improve your quality of life. 
• Oncology social workers specially trained to provide counseling, connect you with community and medical resources, assist with transportation and housing and coordinate care after discharge.
• Our registered dietitian nutritionists offer individualized nutrition support for patients whose efforts to optimize their nutrition may be affected by cancer symptoms or treatment side effects.
• Referrals and professional care from experts in psychology, psychiatry and emotional health, including individual and family counseling to help with the emotional challenges of cancer.
• Physical rehabilitation and occupational therapy services, including wound care, voice therapy and swallowing therapy, lymphedema therapy, balance and vestibular rehabilitation, yoga and more.
• Scripps Center for Integrative Medicine for patients interested in mind-body healing through acupuncture, biofeedback, herbal nutrition, massage therapy, integrative cancer care and more.
• Nondenominational spiritual care offered by our chaplains to help coordinate spiritual care with your own clergy, rabbi or spiritual advisor.
• Visiting patient services if you reside beyond San Diego and want help arranging appointments or learning more about short-term lodging.

For the full spectrum of offerings, please visit our cancer patient support services section.