Bile duct cancer

Bile duct cancer (also referred to as cholangiocarcinoma, cholangioma, or cholangiosarcoma) is a rare cancer involving the bile ducts, the structures within the gastrointestinal (GI) tract that carry bile. Bile is a digestive fluid excreted by the liver that breaks down fats so they can be absorbed by the body. Bile flows through the bile ducts into the intestine.

There are two types of bile duct cancer:

• Intrahepatic cancer affects bile ducts inside the liver.
• Extrahepatic cancer affects bile ducts outside the liver.

While bile duct cancer accounts for only about 3 percent of all gastrointestinal cancers, the cancer specialists at Penn Medicine are experienced in treating it. Our gastrointestinal cancer care teams use the most advanced techniques for diagnosing bile duct cancer and actively research better and more precise ways to detect it. Our multidisciplinary team of gastroenterologists, surgeons, medical oncologists, and radiation oncologists offer compassionate and individualized care for even the most complex and unusual cases.

Most symptoms from cholangiocarcinoma arise from an obstruction within the bile ducts. Typical symptoms are related to a blockage or narrowing of the bile ducts and may include:

• Abdominal pain, particularly in the upper right portion of the abdomen
• Dark urine
• Fever
• Gray- or clay-colored stool
• Itchy skin
• Jaundice or yellowing of the skin and whites of the eyes
• Weight loss without dieting

There are some risk factors that may increase the chance of developing cholangiocarcinoma, though not everyone with these risk factors will get bile duct cancer. Older age and family history may be risk factors, while other factors include:

• Chemical exposure: Exposure to the chemical thorotrast, a form of thorium dioxide, has been linked to the development of cholangiocarcinoma. Thorotrast was banned in the United States in the 1950s, but its effects may show up decades after exposure.
• Chronic liver disease: People with chronic liver disease such as hepatitis B or hepatitis C, alcoholic liver disease, or cirrhosis from other causes may be at an increased risk.
• Congenital liver abnormalities: Choledochal cysts such as Caroli’s diseases have been associated with an increased risk for bile duct cancer.
• Inflammatory diseases of the bile ducts: People with inflammatory diseases of the bile ducts such as ulcerative colitis and primary sclerosing cholangitis (PSC) may be at increased risk for developing bile duct cancer.
• Parasitic liver disease: Parasitic liver disease, such as an infection with the Chinese liver fluke, may increase the risk for developing cholangiocarcinoma.

If you believe you may be at risk, ask your doctor about consulting with a cancer risk assessment specialist at Penn Medicine to help identify your risk and detect bile duct cancer early. While most risk factors for bile duct cancer cannot be prevented, such as pre-existing conditions, there are some things you can do to prevent it. For example, you can maintain a healthy weight, stop smoking, and limit alcohol usage. By working with a risk assessment specialist, you can take preventive steps to manage your risk.

Staging is a way of recording the size and growth of bile duct cancer and determining the best care plan for treatment. By understanding the stage of cancer, you and your team can make the most informed treatment decisions. We use advanced genetic and molecular profiling tests to evaluate and stage bile duct cancer, and our expert pathologists assess every tumor.

The stages of bile duct cancer include:

• Stage 0: Cancerous tissues are in the lining of the bile duct only and have not spread to lymph nodes or other organs. This stage is also called carcinoma in situ.
• Stage I: In Stage IA, cancer is found in the bile duct only. In Stage IB, cancer has spread through the wall of the bile duct.
• Stage II: In Stage IIA, cancer has spread to the liver, gallbladder, pancreas, or the right or left branch of the hepatic artery or the right or left branch of the portal vein. In Stage IIB, cancer has spread to nearby lymph nodes and is found in the bile duct; has spread through the wall of the bile duct; or has spread to the liver, gallbladder, pancreas, or right or left branches of the hepatic artery or portal vein.
• Stage III: Cancer has spread to the main portal vein or to both the right and left branches of the portal vein; cancer has spread to the hepatic artery; or cancer has spread to other nearby organs and tissues.
• Stage IV: A cancerous tumor has spread to distant organs.

A physician may also classify the disease in these ways:

• Local: Cancer is limited to within the bile duct and can be completely removed surgically. There’s no evidence of any spread to areas outside of the bile duct.
• Locally advanced: The cancer is confined to the area around the bile duct, but does affect nearby organs, arteries, or veins. There is no evidence of spreading to any distant areas of the body.
• Metastatic: The tumor has spread beyond the bile duct area to reach distant parts of the body. It’s unlikely that surgery can remove the cancer.

Bile duct cancer surgeries are complex and uncommon operations. As a high-volume center, our surgeons are among the most experienced in the southeastern Pennsylvania and New Jersey regions performing these complicated procedures. They routinely use the most advanced techniques for diagnosing bile duct cancer and actively research better and more precise ways to detect and treat it. Other centers in the region regularly refer patients to us because we can often treat even the most complicated cases that others may consider inoperable.

When you come to Penn Medicine, you can expect:

• Elite surgeons: Surgeries to treat bile duct tumors may involve other nearby organs, such as the liver and pancreas. Our GI surgeons are among the most experienced in the country in these procedures. Their skill leads to better outcomes for our patients and more options for hard-to-treat cancers.
• Complete care: Cholangiocarcinoma treatment often requires more than one therapy or surgery. In our weekly GI tumor board meetings, bile duct cancer experts from multiple medical disciplines come together to discuss treatment approaches and difficult cases. Together, they ensure your care plan is comprehensive and informed by the latest treatments.
• Outstanding outcomes: Our patients experience low rates of complications and excellent results after bile duct cancer surgery. Even patients with more challenging cancers, including advanced cholangiocarcinoma, experience these good outcomes.
• Advanced treatments: You’ll have access to advanced cholangiocarcinoma treatments not available at most centers in the southeastern Pennsylvania and New Jersey regions. These treatments include liver transplants, proton therapy, and hepatic artery infusion pumps that deliver concentrated doses of chemotherapy directly into your liver to treat advanced tumors.
• Clinical trials: Our clinical trial program gives patients early access to breakthrough therapies and provides additional options for inoperable tumors.
• Ongoing follow-up and support: Your treatment doesn’t end after surgery. We help protect your long-term health by following you closely after treatment ends. Your doctor may recommend periodic screenings or check-ups to detect changes that require treatment. Supportive therapies, including palliative care and counseling, help manage additional impacts to your health and wellness. Our cancer nurse navigators serve as a resource throughout your care journey to help you easily get the care you need.