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What is bile duct cancer?

Bile duct cancer, also known as cholangiocarcinoma, is a type of cancer that starts in a bile duct. It can start in any part of the bile duct system.

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Your liver makes a fluid called bile that is stored in your gallbladder. Bile ducts are thin tubes that carry bile from your liver and gallbladder to the small intestine. There, bile helps break down fat from the food you eat. Bile also carries away waste products that your liver filters out of your blood. ?

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The bile duct system has different parts. ?

Inside your liver, the bile duct system starts as many tiny tubes called ductules. These ductules join to form small ducts, which merge into larger ducts. Eventually, they form the left and right hepatic ducts. The left and right hepatic ducts leave the liver and join to form the common hepatic duct.

Bile ducts inside the liver are called intrahepatic bile ducts. Bile ducts located outside the liver are called extrahepatic bile ducts.

The gallbladder is a small organ below the liver that stores bile. A tube called the cystic duct connects the gallbladder to the common hepatic duct. These come together to form the common bile duct. The common bile duct passes through part of the pancreas before it joins with the pancreatic duct and empties into the duodenum (first part of the small intestine) at the Ampulla of Vater.

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Types of bile duct cancers by location

Bile duct cancer (cholangiocarcinoma) can start in any part of the bile duct system. Depending on where the cancer starts, it is either an intrahepatic cholangiocarcinoma or an extrahepatic cholangiocarcinoma.

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Intrahepatic cholangiocarcinoma

Intrahepatic bile duct cancers start in the smaller bile duct branches within the liver. They make up about 10% of all bile duct cancers. Sometimes, intrahepatic bile duct cancers are confused with hepatocellular carcinoma, a cancer that starts in the liver cells. But these are two different types of cancer, and they are treated differently.

Extrahepatic cholangiocarcinoma

There are two types of extrahepatic cholangiocarcinoma: perihilar duct cancer and distal duct cancer.

Perihilar bile duct cancers are found in the hilar region of the bile duct system. The hilar region is where the left and right hepatic ducts leave the liver and join to form the common hepatic duct. Perihilar duct cancers are also called Klatskin tumors.

Distal duct cancers are found along the common bile duct. The common bile duct starts where the common hepatic duct joins with the cystic duct from the gallbladder. It extends through the pancreas and into the small intestine at the Ampulla of Vater.

Cancers that start in different parts of the bile ducts can cause different symptoms.

Types of bile duct cancer by cell type

Bile duct cancers can also be divided into types based on how the cancer cells look under the microscope.

Nearly all bile duct cancers are cholangiocarcinomas. This is a type of adenocarcinoma, which is a cancer that starts in gland cells. Cholangiocarcinomas start in the gland cells that line the inside of the ducts.

Other types of bile duct cancers are much less common. These include sarcomas, lymphomas, and small cell cancers. Our information does not cover these rare types of bile duct cancer.

Benign bile duct tumors

Not all bile duct tumors are cancer. For example, bile duct hamartomas and bile duct adenomas are types of benign (noncancerous) tumors.

Other cancers in the liver

The most common type of cancer that starts in the liver is hepatocellular carcinoma. This type of cancer starts in the main cells that make up the liver. It is more common than cholangiocarcinoma.

Cancers that start in other organs, such as the colon or rectum, can sometimes spread (metastasize) to the liver. These metastatic cancers are not true liver cancers. For example, colorectal cancer that has spread to the liver is still colorectal cancer, not liver cancer.

The treatment and outlook for cancer that metastasizes to the liver is not the same as for a cancer that starts in the liver. For this reason, it¡¯s important to know whether a tumor in the liver started in bile ducts (cholangiocarcinoma), if it started in the liver tissue itself (hepatocellular carcinoma), or if it started in another organ and has spread to the liver.