Cholangiocarcinoma

Cholangiocarcinoma, or bile duct cancer, is a rare cancer that’s a part of the biliary tract cancers, and it makes up 2-3% of all GI cancer cases. Cholangiocarcinoma is often caused by unknown reasons, and it rarely has symptoms until someone has advanced disease. For this reason, many of these cancers are aggressive and hard to treat.

There’s hope in biomarker testing for cholangiocarcinoma patients. In about 40-50% of patients, there is at least one "actionable" biomarker, meaning there’s an FDA-approved drug that may be incorporated into your treatment plan to target your specific mutations. (This will depend on if you’re receiving first-line or second-line therapy, what the FDA approved the treatment for, your overall condition, etc.)

There are multiple targeted therapies and immunotherapies being utilized in treatment plans, and several more treatment combinations are being studied in ongoing clinical trials, which means the treatment landscape for cholangiocarcinoma is continually changing. 

What is cholangiocarcinoma?

Cholangiocarcinoma forms in the network of small ducts that carry bile from the liver to the gallbladder to the small intestine. Bile duct cancer can form in the main bile ducts outside the liver (extrahepatic) or within the liver (intrahepatic).

Types of cholangiocarcinoma

Most bile duct cancers are adenocarcinomas, cancer that forms in glands that line internal organs and make mucus and other fluids. Ninety percent of cholangiocarcinomas form outside the liver (extrahepatic). 

There are several types of cholangiocarcinoma:

• Extrahepatic perihilar bile duct cancer, also known as a Hilar or Klatskin tumor, or perihilar cholangiocarcinoma: Starts where the right and left bile ducts connect and leave the liver (an area called the hilum). This is the most common form, making up 60-70% of cholangiocarcinomas.
  
• Extrahepatic distal bile duct cancer, also known as distal cholangiocarcinoma: Starts after the right and left hepatic bile ducts have joined to form the common bile duct outside the liver (near the small intestine). These make up 20-30% of cholangiocarcinomas.
• Intrahepatic bile duct cancer, also known as intrahepatic cholangiocarcinoma (ICC/iCCA): About 10% of all cases. Starts inside the bile ducts inside the liver. This form of cancer is increasing worldwide, and is the second-most frequent type of primary liver cancer. In some very rare cases, some tumors may have tiny features of both liver cancer and cholangiocarcinoma. 

Staging bile duct cancer

If you have cholangiocarcinoma, you need to know your stage. This helps you understand where the cancer is now, the best treatment plan for your unique situation, and your outlook.

Intrahepatic, distal, and perihilar cholangiocarcinoma is staged slightly differently, but it’s all based on the tumor's location, size, and spread. These cancers are staged on a scale from 0-4 (many researchers use roman numerals 0-IV)  using the TNM staging system. 

To see the intricacies of each stage of cholangiocarcinoma, visit the staging information by our members The Cholangiocarcinoma Foundation.

What causes bile duct cancer?

Risk factors put you at a higher risk of getting cancer. Some risk factors can be controlled with lifestyle changes, and others cannot. Older age, smoking, and obesity increase your risk of many cancers.

Just because you have a risk factor, that doesn’t mean you will get cancer. 

These are scenarios researchers have found that can increase your risk: 

• Inflammation of the bile ducts (primary sclerosing cholangitis)
• Chronic liver disease and cirrhosis (from hepatitis B or C, alcohol, or fatty liver disease)
• Bile duct cysts at birth 
• Bile duct stones/gallstones
• Parasites
• Type 1 or type 2 diabetes
• Certain inherited conditions, like cystic fibrosis and Lynch syndrome
• Exposure to toxins (rubber plants and automotive factories)

Preventing bile duct cancer

You can’t prevent cholangiocarcinoma, but you can reduce your risk. Protect yourself from viruses like hepatitis B or C.

As with every cancer, there are steps you can take to adopt a healthy lifestyle and reduce your overall cancer risk, such as maintaining a healthy weight, not smoking, and limiting or eliminating alcohol. 

Learn more about cancer prevention from the American Institute for Cancer Research.

How common is cholangiocarcinoma? 

Cholangiocarcinoma is a rare disease.

About 8-10,000 people are diagnosed each year. 

Two-thirds of patients are 65 or older when diagnosed; the average age of extrahepatic cholangiocarcinoma is 72 and for intrahepatic cholangiocarcinoma it’s 70. It’s slightly more common in men than women.

What are my odds of surviving it?

Like many other rare GI cancers, cholangiocarcinoma is often caught at late stages, when it’s most difficult to treat. Doctors use what’s called “survival statistics” to understand the likelihood of a patient beating the disease.

Detailed survival statistics are based on where the cancer is located and if it has spread:

Localized

The cancer hasn’t spread

• 25% of intrahepatic cancer patients are alive five years after diagnosis 
• 19% of extrahepatic cancer patients are alive five years after diagnosis 

Regional

The cancer spread to nearby structures or lymph nodes

• 12% of intrahepatic cancer patients are alive five years after diagnosis 
• 20% of extrahepatic cancer patients are alive five years after diagnosis 

Distant

The cancer has spread to other organs

• 3% of intrahepatic cancer patients are alive five years after diagnosis 
• 2% of extrahepatic cancer patients are alive five years after diagnosis 

What types of tests should I expect to undergo?

Doctors will likely use these tests to diagnose cholangiocarcinoma:

• Physical exam
• Blood tests checking bilirubin, liver enzymes, and tumor markers
• Ultrasound

Endoscopy, MRI, PET scan, PTC, and biopsy may also be used to identify and diagnose bile duct cancer.

Ask your doctor about these additional tests—ideally before you begin treatment.

• Biomarker testing
• Genetic testing

I’m facing a diagnosis: What are my next steps?

If you’re facing a diagnosis, the GI Cancers Alliance is here for you. 

If you’re newly diagnosed, work with your doctor to create a treatment plan. It’s important to find an expert who specializes in cholangiocarcinoma and visit a major cancer center if possible.

Most cholangiocarcinoma treatment plans include:

• Surgery (possible liver transplant)
• Chemotherapy 
• Radiation 
• Targeted therapy
• Immunotherapy

We strongly encourage you to get a second opinion from another trusted doctor so you feel confident moving forward.