Cholangiocarcinoma (Bile Duct Cancer)

Cholangiocarcinoma is a rare cancer that starts in your bile ducts. Bile ducts are thin tubes that bring bile (a fluid that helps you digest fats) from your liver and gallbladder to your small intestine.

Cholangiocarcinoma is an aggressive cancer, which means it spreads fast. Most people receive a cholangiocarcinoma diagnosis after it’s already spread outside of their bile ducts. At this point, bile duct cancer is difficult to treat, and the prognosis (chance of recovery) is usually poor.

Experts are continually researching and developing new treatments that can slow cancer spread and improve the outlook associated with cholangiocarcinoma.

What are the types of bile duct cancer?

There are three types of cholangiocarcinoma:

• Intrahepatic cholangiocarcinoma is bile duct cancer inside your liver.
• Perihilar (hilar) cholangiocarcinoma is bile duct cancer in your hilum. The hilum is the area just outside your liver where the smaller bile ducts from inside your liver merge to form a larger duct called the common hepatic duct. It’s the most common form of cholangiocarcinoma. Another name for perihilar cholangiocarcinoma is a Klatskin tumor.
• Distal cholangiocarcinoma is bile duct cancer that starts outside your liver, in the ducts closer to your small intestine.

Perihilar cholangiocarcinoma and distal cholangiocarcinoma are also known as extrahepatic bile duct cancers because they form outside your liver (“extra”-hepatic) instead of inside your liver (“intra”-hepatic).

How common is this condition?

Cholangiocarcinoma is rare. About 8,000 people in the United States develop this cancer each year. It’s most common in people around age 70.

Worldwide, cholangiocarcinoma is more common in Southeast Asia. Bile duct cancer is a complication of clonorchiasis, a chronic (long-term) infection associated with a Chinese liver fluke parasite.