%0 Journal Article
%T Cholangiocarcinoma: Biology, Clinical Management, and Pharmacological Perspectives
%A Rocio I. R. Macias
%J ISRN Hepatology
%D 2014
%R 10.1155/2014/828074
%X Cholangiocarcinoma (CCA), or tumor of the biliary tree, is a rare and heterogeneous group of malignancies associated with a very poor prognosis. Depending on their localization along the biliary tree, CCAs are classified as intrahepatic, perihilar, and distal, and these subtypes are now considered different entities that differ in tumor biology, the staging system, management, and prognosis. When diagnosed, an evaluation by a multidisciplinary team is essential; the team must decide on the best therapeutic option. Surgical resection of tumors with negative margins is the best option for all subtypes of CCA, although this is only achieved in less than 50% of cases. Five-year survival rates have increased in the recent past owing to improvements in imaging techniques, which permits resectability to be predicted more accurately, and in surgery. Chemotherapy and radiotherapy are relatively ineffective in treating nonoperable tumors and the resistance of CCA to these therapies is a major problem. Although the combination of gemcitabine plus platinum derivatives is the pharmacological treatment most widely used, to date there is no standard chemotherapy, and new combinations with targeted drugs are currently being tested in ongoing clinical trials. This review summarizes the biology, clinical management, and pharmacological perspectives of these complex tumors. 1. Primary and Metastatic Liver Cancer Primary liver cancer accounts for approximately 10¨C12% of deaths due to cancer. Although the incidence of this group of cancers is lower than 6% of new cancers diagnosed each year worldwide, the prognosis is usually very poor. The most frequent of these tumors are adenocarcinomas, which include hepatocellular carcinoma (HCC) derived from parenchymal cells¡ªaccounting for almost 85% of liver adenocarcinomas and cholangiocarcinoma (CCA), derived from biliary epithelial cells and accounting for the remaining 15%. Other rare primary liver tumors include hemangiosarcoma, derived from endothelial cells, and hepatoblastoma, derived from embryonic or fetal hepatocyte precursors. Even less frequent primary liver cancers are fibrosarcoma and lymphosarcoma. It should also be considered that the liver is highly vulnerable to tumor invasion from extrahepatic metastasis. The large size of the liver, its abundant blood supply, and its double-source vascularization explain why it is the second most common seat of metastasis after lymph nodes. Among the tumors that most frequently metastasize to the liver are colorectal cancer, breast cancer, melanoma, and lung cancer. 2.
%U http://www.hindawi.com/journals/isrn.hepatology/2014/828074/