Klatskin Tumor (Hilar Cholangiocarcinoma)

What is Klatskin tumor (hilar cholangiocarcinoma)?

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.

How rare is a Klatskin tumor?

This kind of tumor is very rare, affecting an estimated 1 in 100,000 people.

What are Klatskin tumor symptoms?

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:

• Abdominal pain.
• Fatigue.
• Nausea.
• Unexplained weight loss.
• Yellow skin or yellow whites of your eyes from jaundice.

Managing symptoms may be challenging, so don’t hesitate to ask your healthcare provider for help.

What causes Klatskin tumor?

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:

• Alcohol use disorder.
• Choledocholithiasis.
• Chronic pancreatitis.
• Clonorchiasis (infection with a Chinese liver fluke parasite).
• Primary sclerosing cholangitis.
• Viruses, including hepatitis B and hepatitis C.

What are the complications of hilar cholangiocarcinoma?

This condition may cause serious complications like:

• Blood clots: The tumor may spread into major blood vessels and create blood clots.
• Extrahepatic cholestasis: This happens when a tumor blocks a large bile duct outside your liver.
• Metastatic bile duct cancer: Klatskin tumors may spread to your liver from the bile duct outside your liver, as well as to your lungs and bones.

How is Klatskin tumor diagnosed?

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

Blood tests may include:

• Tumor markers: These tests check for substances in your blood that may be a sign of Klatskin tumor. For example, they may test the level of carbohydrate antigen (CA) 19-9. High levels of CA 19-9 may be a sign of the condition.
• Liver function tests: These may include bilirubin tests and high liver enzyme tests.

Imaging tests

Some imaging tests are also a way to obtain tissue for review by a medical pathologist. These may include:

• Magnetic resonance imaging (MRI).
• Endoscopic retrograde cholangiopancreatography (ERCP).
• Magnetic resonance cholangiopancreatography (MRCP).

Stages

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:

• Stage 0: The tumor is in the inner layers of your bile duct, but it hasn’t spread into deeper layers, your lymph nodes or other areas of your body.
• Stage I: There’s cancer in your bile duct wall, but it hasn’t spread outside your bile duct.
• Stage II: The tumor has spread to nearby fatty tissue or into nearby liver tissue.
• Stage IIIA:: There’s cancer spreading into the branches on one side (left or right) of your liver’s main blood vessels.
• Stage IIIB: The tumor has spread to both sides of the main blood vessels in your liver or into other bile ducts on one side of your liver and a main blood vessel on the other side.
• Stage IIIC: Cancer is in one to three nearby lymph nodes.
• Stage IVA: The tumor has spread to four or more lymph nodes.
• Stage IVB: Cancer is now in your lungs, bones or more distant parts of your liver.

What are treatments for Klatskin tumor?

Treatments will vary depending on your situation, but surgery is a common front-line or initial treatment for this condition. Surgery may be:

• Biliary drainage: Providers may do this procedure to drain extra bile from your liver, which eases symptoms like jaundice.
• Extended hepatectomy: People with early-stage tumors may have surgery to remove part of their livers (hepatectomy), their bile duct, liver, gallbladder and nearby lymph nodes.
• Liver transplantation: The only cure for this condition is removing the cancerous tumor(s), which may mean removing your liver and having a liver transplant.

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:

• Immunotherapy, like immune checkpoint inhibitors that work by helping your immune system to fight cancer.
• Targeted therapy, like fibroblast growth factor receptor (FGFR) inhibitors or IDH inhibitors. FGFR inhibitors block cancerous cell growth so the cells die. IDH inhibitors block an abnormal protein that affects normal cell growth.

Treatment complications and side effects

Hepatectomies and liver transplantations are major surgeries with significant potential complications, such as:

• Ascites: This is fluid buildup in your belly.
• Bile leakage: A hepatectomy may damage bile ducts in your liver so the ducts leak excess bile into your liver.
• Deep vein thrombosis: This is a blood clot in veins deep in your body that may develop after surgery. It’s a complication of liver transplantations and hepatectomies.
• Infection: You may develop an infection in the surgical site, or your urinary tract or lungs.
• Kidney failure: Sometimes, liver surgery affects your kidneys, causing them to stop working.
• Liver failure: This can happen if you have surgery to remove part of your liver (hepatectomy).
• Organ rejection: Your body may reject a transplanted liver.

Common side effects of radiation therapy and chemotherapy include:

• Fatigue.
• Hair loss.
• Nausea and vomiting.

What is the survival rate for Klatskin tumor?

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.

Other survival rates are:

As you think about survival rates, try to keep two things in mind:

• These are estimates: They’re based on the experiences of other people, and your situation may be very different. For example, your overall health and age may affect your estimated survival rate.
• The numbers reflect the past: Survival rates reflect the recent past, not the present. Experts measure survival rates every five years, so current rate information doesn’t account for the impact of any new treatments.

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.

How do I take care of myself?

Maintaining your quality of life throughout diagnosis, treatment and beyond is the best way to take care of yourself. Here are some suggestions:

• Eat well: A diet that emphasizes lean proteins and full-fat food can help with Klatskin tumor symptoms and treatment side effects.
• Manage misinformation: Hilar cholangiocarcinoma is a rare condition. You may not know much about it, and trying to learn more may make you feel anxious, afraid and overwhelmed. But knowledge is power. Do the best you can and ask your care team about any concerns or questions you have.
• Support your mental health: If you have this condition, you have a rare cancer that often requires surgery and long-term care that can take a toll on your mental health. Talk to your care team about programs, treatments and services that may help.
• Consider cancer survivorship programs: These programs emphasize health, wellness and quality of life as you navigate cancer.
• Ask about palliative care: Palliative care for bile duct cancer may include medication to ease nausea, pain control and maintain the flow of bile where a tumor may block it.

When should I see my healthcare provider?

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.

What questions should I ask my healthcare provider?

You may have read a lot about Klatskin tumor and have questions about your situation. Here are some suggestions:

• How do you know I have this kind of cancer?
• What is the cancer stage?
• What are my treatment options?
• Is there a cure?
• Are there clinical trials I should consider?