Article Abstract
Introduction:
Cholangiocarcinoma (CCA) is a cancer arising from the epithelium of intrahepatic or extrahepatic bile ducts caused by the malignant transformation of hepatic biliary cholangiocytes [1][2]. Cholangiocarcinoma can occur anywhere from the small peripheral hepatic ducts to the distal common bile duct. The three primary types of CCA and their relative frequency are intrahepatic (10%), distal (40%) and perihilar (50%), the latter being confined to the larger bile ducts in the hepatic hilum. Mixed hepatocellular cholangiocarcinomas have recently been described in which CCA and hepatocellular carcinoma are found in the same nodule. Cholangiocarcinoma primarily arises from the biliary epithelium in the case of extrahepatic cholangiocarcinoma while hepatic progenitors are believed to play a role in intrahepatic CCA. Cholangiocarcinomas have a dense stromal component that result from the recruitment of fibroblasts, remodeling of the extracellular matrix, altered immune cell migration, and angiogenesis. The tumor stroma surrounds the malignant ducts and glands and comprises most of the tumor mass.
Article Citation:
Author Speaks
“This is the first review article on the current treatment options for cholangiocarcinoma, and we hope it will help bring cholangiocarcinoma to the forefront of drug development. We dedicate this work to all the patients, caregivers, volunteers and the board members of the Cholangiocarcinoma Foundation who have worked countless hours to improve the quality of life and identify new treatment options for patients with this devastating disease.”
– Dr. Carl S. Hornfeldt (Author)