Cholangiocarcinoma (Bile Duct Cancer)

Also known as bile duct cancer, cholangiocarcinoma develops in the tubes that carry digestive fluid (bile) from the liver, where it is produced, to the gallbladder, where it is stored, to the small intestine, where it breaks down fat in digested food. Much like most other tumors, cholangiocarcinoma begins with cellular DNA changes that cause the cells to rapidly grow and divide. The excess cells then build up, bind together and form masses.

Cholangiocarcinoma is categorized based on its site of origin in the bile duct system:

  • Perihilar bile duct cancer develops at the point where the left and right hepatic ducts exit the liver (hilum) and join together to form the common hepatic duct.
  • Distal cholangiocarcinoma forms outside the liver near the small intestine.
  • Intrahepatic bile duct cancer originates in the small bile duct branches inside the liver.

What Causes Cholangiocarcinoma?

The precise causes of bile duct cancer are not yet well understood. Scientists continue to explore the seemingly random events behind the cellular DNA mutations that lead to its development.

What Are the Symptoms of Cholangiocarcinoma?

Early-stage cholangiocarcinoma is often silent, but symptoms can develop if a growing tumor blocks a bile duct and prevents bile from exiting the liver. As a result, bile may back up into the bloodstream, then travel to and settle in different areas of the body.

Signs of a biliary obstruction can include:

  • Jaundice – Bile contains bilirubin, a greenish-yellow chemical that can cause a noticeable yellowing of the whites of the eyes and the skin. 
  • Itchy skin – In the skin, high levels of bilirubin can cause intense itchiness. 
  • Dark-colored urine – Excess bilirubin in the blood may be eliminated in the urine, causing it to darken in color. 
  • Light-colored stool – When bile passes freely from the liver to the small intestine, it aids in the breakdown and absorption of fats and naturally darkens the stool. If bile cannot reach the small intestine, excess fat will be eliminated in the stool, causing it to appear greasier, bulkier and lighter in color than usual. 
  • Abdominal pain – A biliary obstruction may cause discomfort below the ribs on the right side of the abdomen.

How Is Cholangiocarcinoma Diagnosed?

If bile duct cancer is suspected, a physician will typically order one or more diagnostic tests. Blood work and liver function tests can be used to measure the level of bilirubin and other substances, such as albumin, liver enzymes and tumor markers, in the blood.

Imaging technologies such as endoscopic ultrasound can produce highly detailed images to help a physician evaluate suspicious masses and check for a biliary obstruction. For a more direct view of a bile duct or a possible tumor, a physician may insert a small, thin tube with a light and camera at the end (endoscope) through a small incision or a natural body opening.

If the initial diagnostic test results suggest cholangiocarcinoma, a physician may perform a biopsy or treat the tumor as cancerous and proceed directly to surgery.

How Is Cholangiocarcinoma Treated?

Surgery is the main form of treatment for a resectable bile duct tumor. Some patients also undergo chemotherapy, radiation therapy, photodynamic therapy and/or a biliary drainage procedure.

Tampa General Hospital is a recognized leader and innovator in the treatment of cholangiocarcinoma and other liver cancers. Our liver and hepatobiliary oncologists emphasize personalized care and offer the very latest options, including hyperthermic intraperitoneal chemotherapy (HIPEC) and liver transplantation.