Cholangiocarcinoma can begin in the green ducts (at right) that collect bile being made in the liver, termed intrahepatic cholangiocarcinoma. If the cancer originates in the ducts outside of the liver that deliver bile to the small intestine, then it is termed extrahepatic cholangiocarcinoma.
Intrahepatic cholangiocarcinoma makes up only about 10% of all cholangiocarcinomas, yet it is the second most common malignancy arising from the liver. Only a minority of patients (15%) present with resectable disease, with a median survival of less than 3 years.
Complete surgical resection remains the only option for cure with an estimated median survival ranging from 27 to 36 months.
For cases of intrahepatic cancers that cannot be surgically removed, a liver transplantation may be an option. In some cases, a liver transplant might even cure the cancer. Finally, radiation and chemotherapy are also treatment options.
Buettner S, LA van Vugt J, IJzermans JNM, Koerkamp BG. Intrahepatic cholangiocarcinoma: current perspectives. Onco Targets Ther. 2017: 10: 1131-1142.
For more information, visit the Genetic and Rare Diseases (GARD) Information Center website on ICC (https://rarediseases.info.nih.ov/diseases/6042/intrahepatic-cholangiocarcinoma)