What is cholangiocarcinoma?
Cholangiocarcinomas (CCC) are tumors that originate in the bile ducts – the tubes that transport bile from the liver to the gallbladder and on to the small intestine. These tumors are also referred to as hepatic duct tumors.

One-third of all liver and biliary passage tumors are in the intrahepatic ducts, which pass through and drain bile from the liver, and extrahepatic ducts, which move bile outside the liver. Tumors can start in large collecting ducts, including the bile ducts and hepatic ducts, or in smaller ducts throughout the organ.

What causes CCC?
Although we don’t know the causes of all bile duct cancer, there are some diseases and conditions associated with bile duct tumors. They include fluke infections of the liver; gallstones, which may result in persistent irritation of the ducts; ulcerative colitis; and primary sclerosing cholangitis, or inflammation and scarring of the bile ducts. Other, less commonly associated diseases include cystic liver disease and Caroli’s disease, an inherited disease in which bile ducts in the liver are enlarged and may lead to irritation, infection and/or gallstones.

What are the signs and symptoms of CCC?
In many cases, patients with a malignant tumor in a hepatic duct have symptoms similar to those of advanced primary liver cancer. However, these tumors often cause blockage of the ducts, which can lead to painless, new-onset jaundice – a yellowish coloring of the skin that results from excess bile.

How is CCC diagnosed?
Hepatic duct cancer may be diagnosed in one or more of the following ways. A CT scan prompted by complaints of gastrointestinal pain or discomfort may reveal duct tumors. A CT scan may also reveal a dilated bile duct. Or, as part of an endoscopic examination of the gastrointestinal tract, a gastroenterologist may notice a tumor or a narrowing of a duct that could indicate the presence of a tumor. This examination is called endoscopic retrograde cholangiopancreatography (ERCP). If an ERCP exam produces evidence of a bile duct tumor, the gastroenterologist may take sample of tissue during the procedure. The sample will then undergo a biopsy.

Once diagnosed, how is CCC evaluated?
Once liver duct cancer is suspected or diagnosed, a patient may undergo additional evaluations – including cholangiography (in which a dye is injected and its movement analyzed), enhanced CT scans, an angiogram (which helps determine blood vessel involvement and blood flow to the tumor) and an MRI cholangiogram. Together, such tests yield valuable information about the location and size of the tumor, how close it is to major hepatic blood vessels, whether or not the tumor is spreading, and overall liver function.

Knowing the location of the tumor is critical in determining an optimal course of treatment. Similarly, blood tests to measure bilirubin levels may be needed to determine whether or not a tumor is blocking the duct. If tests indicate increased bilirubin and duct blockage, our specialists may place a stent, or tube, in the duct to relieve the blockage and return bilirubin levels to normal.

Finally, patients who will be treated by chemotherapy may also need to undergo a hearing test and a MUGA heart scan.

Contact us
To schedule an appointment with a Jefferson physician call 1-800-JEFF-NOW or click here.

To contact the Liver Tumor Program team at the Kimmel Cancer Center at Jefferson, please call 215-503-8752 and/or fax your records to 215-503-8755. We aim to schedule your first clinic visit within two weeks of your diagnosis being made and records being received.