Klatskin tumor (hilar cholangiocarcinoma) is a rare cancer where there are aggressive tumors on your bile duct. The tumors are highly treatable, especially when healthcare providers detect and do surgery to remove tumors before they spread. Providers have other treatments that help to ease symptoms. They may also suggest clinical trials.
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Hilar cholangiocarcinoma is a form of extrahepatic bile duct cancer. In this condition, you have cancerous tumors on part of your bile duct, outside of your liver. These tumors are known as Klatskin tumors. Healthcare providers may be able to cure this condition if they detect and treat tumors before they spread. Surgery, including liver transplant, is the most common treatment. Providers have other treatments that ease symptoms. And researchers are doing several clinical trials to evaluate new treatments.
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This kind of tumor is very rare, affecting an estimated 1 in 100,000 people.
This condition affects your hilum. That’s the area right outside your liver where your left and right bile ducts come together to form your common bile duct. Cancerous tumors on your bile ducts block the flow of bile. When that happens, you may have symptoms like:
Managing symptoms may be challenging, so don’t hesitate to ask your healthcare provider for help.
Hilar cholangiocarcinoma develops when normal cells mutate (change) and become abnormal cells. These abnormal cells divide and multiply uncontrollably, eventually creating tumors. Researchers don’t know what causes the mutation. They do know some conditions and activities increase your risk, like medical conditions and genetic mutations that happen after you’re born.
Medical conditions that increase the risk of Klatskin tumors include:
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This condition may cause serious complications like:
If your healthcare provider thinks you may have hilar cholangiocarcinoma, they’ll do a physical examination that focuses on your abdomen (belly). They’ll look for signs of fluid buildup, lumps or tenderness in your belly. They’ll examine your skin and the whites of your eyes for jaundice.
Your provider may do blood tests, imaging tests and tests that allow them to see inside your body to check on what’s happening to your bile ducts. They may do genetic testing to look for specific genetic mutations.
Blood tests may include:
Some imaging tests are also a way to obtain tissue for review by a medical pathologist. These may include:
If you have hilar cholangiocarcinoma, your provider will use cancer staging information to plan your treatment and prognosis, or what you can expect after treatment.
Cancer stages are classified by tumor size (T), whether cancer is in your lymph nodes (N) and if it’s metastasized (M). Providers may also consider cancer grade, which is how cancerous cells appear when viewed under a microscope. Klatskin tumor stages are:
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Treatments will vary depending on your situation, but surgery is a common front-line or initial treatment for this condition. Surgery may be:
Providers may use chemoradiation, which combines chemotherapy and radiation therapy, along with surgery or when surgery isn’t an option. Two newer treatments for metastatic Klatskin tumor are:
Hepatectomies and liver transplantations are major surgeries with significant potential complications, such as:
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Common side effects of radiation therapy and chemotherapy include:
If you have this condition, one of your first questions may be about its survival rate. Survival rates estimate the percentage of people with a specific cancer who were alive five years after their diagnosis.
The U.S. National Cancer Institute (NCI) maintains a cancer survival rate database. Instead of tracking five-year survival rates by cancer stage, like stage 0 through stage IV, the database groups cancers and sets stages by tumor location activity. While there isn’t survival rate data available for hilar cholangiocarcinoma, there is data for all bile duct cancers that develop outside of your liver. Overall, 9% of people with extrahepatic bile duct cancer were alive five years after diagnosis.
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Other survival rates are:
Tumor location | Five-year survival rate |
---|---|
Localized tumors: No sign of cancer outside your bile ducts. Localized tumors: No sign of cancer outside your bile ducts. | 23% |
Regional tumors: Cancer has spread outside bile ducts to nearby tissue, organs or lymph nodes. | 9% |
Distant tumors: There’s bile duct cancer in your lungs and other areas of your body. | 3% |
Tumor location | |
Localized tumors: No sign of cancer outside your bile ducts. Localized tumors: No sign of cancer outside your bile ducts. | |
Five-year survival rate | |
23% | |
Regional tumors: Cancer has spread outside bile ducts to nearby tissue, organs or lymph nodes. | |
Five-year survival rate | |
9% | |
Distant tumors: There’s bile duct cancer in your lungs and other areas of your body. | |
Five-year survival rate | |
3% |
As you think about survival rates, try to keep two things in mind:
As you learn more about your condition, be sure to ask your provider to explain survival rates and what the rate information may mean for you.
Maintaining your quality of life throughout diagnosis, treatment and beyond is the best way to take care of yourself. Here are some suggestions:
Ask your provider what changes to expect, like symptoms that get worse or new symptoms. Don’t hesitate to contact them for help managing symptoms like pain or nausea and vomiting. Your provider will have treatments to reduce symptoms that affect your daily and your quality of life.
You may have read a lot about Klatskin tumor and have questions about your situation. Here are some suggestions:
Klatskin tumor (hilar cholangiocarcinoma) is an aggressive type of bile duct cancer. There’s no question that it’s a challenging and serious illness. But data like survival rates don’t tell the whole story about this condition. It’s highly treatable, and researchers are doing clinical trials for new treatments. If you have this condition, your healthcare team will help you manage symptoms and treatment side effects. They’ll also be glad to suggest clinical trials you may want to consider.
Last reviewed on 01/18/2024.
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