Bile Duct Cancer (Cholangiocarcinoma)
Watch Sam Pappas, MD, Medical College of Wisconsin surgical oncologist, answer the question: "What is cholangiocarcinoma?"
Cancers that develop in the bile ducts are known as cholangiocarcinomas. Cholangiocarcinomas can be located in the bile ducts inside the liver (“intrahepatic”), or in ducts outside of the liver (“extrahepatic”).
Several studies suggest an association between cholangiocarcinomas and infection with certain types of liver parasites, particularly in Thailand. Eating undercooked fish can lead to ingestion of these parasites (especially one called Opisthorcis viverrini) which can then lay eggs in the human host’s biliary system. The flukes can cause mechanical irritation as well as giving off chemicals which can promote cancer.
In the West, a common cause of cholangiocarcinoma is primary sclerosing cholangitis, or “PSC.” This is an inflammatory condition thought to be related to abnormalities in the immune system which can cause blockages within bile ducts within the liver.
Up to 40% of patients with PSC can develop cholangiocarcinoma, and cancers in these patients tend to present at a young age, typically between 30 and 50. Patients with PSC also have up to a 90% chance of having ulcerative colitis, an inflammatory disease which affects the colon. This can also increase their risk for colon cancer.
Other rarer causes of cholangiocarcinomas include genetic disorders, including some associated with other cancers like colon cancer, and diseases which cause cysts to develop in the liver. Underlying scarring of the liver, or cirrhosis, can also lead to a 10-fold higher risk of cholangiocarcinoma compared with the general population. The incidence of cholangiocarcinoma associated with cirrhosis is rising in the United States.
Staging of cholangiocarcinoma will help make decisions about what treatments are possible and help predict the potential for survival. Staging describes where the cancer is and whether or not it invades any major structures. It also helps doctors compare results from treatment choices used in different studies. \
Staging Bile Duct Cancer
Cholangiocarcinomas are malignant tumors that arise from the lining of bile ducts. Bile ducts, small tubes that
carry a green substance called bile, arise within the liver and converge to form a single common bile duct, which carries bile from the liver through the pancreas and into the intestine.
Tumors can arise from the bile ducts within the liver (intrahepatic cholangiocarcinoma), the ducts between the liver and the pancreas (extrahepatic cholangiocarcinoma, or Klatskin tumor), or they can develop in the bile duct as it passes through the pancreas (distal common bile duct or periampuallary cholangiocarcinoma). The surgical treatment of tumors of the bile duct is mainly determined by their location.
Cholangiocarcinomas that arise inside the liver, or intrahepatic cholangiocarcinoma, are difficult to detect as they can grow without causing symptoms or signs that are detectable by health care providers. It can be difficult to tell the difference between intrahepatic cholangiocarcinomas and tumors which have spread to the liver from other organs (i.e. colon, stomach, breast) so tests may be done to rule out this possibility.
The surgical treatment of intrahepatic cholangiocarcinoma involves removing the portion of the liver containing the tumor, known has hepatectomy. Surgeons can remove up to 70-80% of a normal person’s liver as a normal healthy liver has the ability to regenerate after a portion of it is removed.
Extrahepatic Cholangiocarcinomas
Extrahepatic cholangiocarcinomas are often referred to as Klatskin tumors. They mainly develop at the site where the bile ducts from the left and right sides of the liver join, known as the hepatic duct confluence. These cancers grow and block off bile ducts. If the bile ducts from both sides of the liver become blocked, bile backs up into the liver and the patient can become jaundiced.
Jaundice
Jaundice is yellowing of the skin and eyes, itching, darkening of the urine and occasionally pale stools. The surgical management of Klatskin tumors involves the removal of the main bile duct and the surrounding lymph nodes, as well as removal of one half to two-thirds of the liver. The side (left or right) of the liver that is removed depends on which side the tumor is mainly situated.
Occasionally surgery for these tumors requires removal of a portion of the portal vein (the vein which drains blood from the intestine to the liver) since these tumors can often grow around this blood vessel.
Tumors that arise in the bile duct as it passes through the pancreas usually become noticed early because the tumor block off the common bile duct. Doctors may relieve the jaundice and open up the bile duct (biliary decompression) by placing a stent in the duct through an endoscope (ERCP) or through the liver (percutaneous transhepatic catheter, PTC).
Surgical treatment of these tumors involves a pancreaticoduodenectomy, or Whipple procedure. This operation involves the removal of the bile duct, part of the pancreas and the first part of the small intestine (duodenum); a portion of the stomach is often removed as well.
Last Updated on 2/20/2012 3:04:07 PM