Table of Contents
ISRN Hepatology
Volume 2014, Article ID 828074, 13 pages
Review Article

Cholangiocarcinoma: Biology, Clinical Management, and Pharmacological Perspectives

1Laboratory of Experimental Hepatology and Drug Targeting (HEVEFARM), University of Salamanca, IBSAL, 37007 Salamanca, Spain
2National Institute of Health Carlos III, CIBERehd, 28029 Madrid, Spain
3Department of Physiology and Pharmacology, Campus Miguel de Unamuno E.D. B-17, University of Salamanca, 37007 Salamanca, Spain

Received 5 December 2013; Accepted 2 January 2014; Published 16 February 2014

Academic Editors: S. DeMorrow, M. G. Mancino, and S. Pinlaor

Copyright © 2014 Rocio I. R. Macias. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.