Review
Copyright ©2009 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. Sep 14, 2009; 15(34): 4240-4262
Published online Sep 14, 2009. doi: 10.3748/wjg.15.4240
Advances in diagnosis, treatment and palliation of cholangiocarcinoma: 1990-2009
Murad Aljiffry, Mark J Walsh, Michele Molinari
Murad Aljiffry, Mark J Walsh, Michele Molinari, Department of Surgery, Queen Elizabeth II Health Science Centre, Dalhousie University, Rm 6-254, Victoria Building, 1278 Tower Road, Halifax, Nova Scotia, B3H 2Y9, Canada
Author contributions: Aljiffry M and Molinari M contributed equally to this work by performing the literature search and by writing the paper; Walsh MJ reviewed and edited the manuscript.
Correspondence to: Michele Molinari, MD, Department of Surgery, Queen Elizabeth II Health Science Centre, Dalhousie University, Rm 6-254, Victoria Building, 1278 Tower Road, Halifax, Nova Scotia, B3H 2Y9, Canada. michele.molinari@cdha.nshealth.ca
Telephone: +1-902-4737624 Fax: +1-902-4737639
Received: August 5, 2008
Revised: July 15, 2009
Accepted: July 22, 2009
Published online: September 14, 2009
Abstract

Several advances in diagnosis, treatment and palliation of cholangiocarcinoma (CC) have occurred in the last decades. A multidisciplinary approach to this disease is therefore recommended. CC is a relatively rare tumor and the main risk factors are: chronic inflammation, genetic predisposition and congenital abnormalities of the biliary tree. While the incidence of intra-hepatic CC is increasing, the incidence of extra-hepatic CC is trending down. The only curative treatment for CC is surgical resection with negative margins. Liver transplantation has been proposed only for selected patients with hilar CC that cannot be resected who have no metastatic disease after a period of neoadjuvant chemo-radiation therapy. Magnetic resonance imaging/magnetic resonance cholangiopancreatography, positron emission tomography scan, endoscopic ultrasound and computed tomography scans are the most frequently used modalities for diagnosis and tumor staging. Adjuvant therapy, palliative chemotherapy and radiotherapy have been relatively ineffective for inoperable CC. For most of these patients biliary stenting provides effective palliation. Photodynamic therapy is an emerging palliative treatment that seems to provide pain relief, improve biliary patency and increase survival. The clinical utility of other emerging therapies such as transarterial chemoembolization, hepatic arterial chemoinfusion and high intensity intraductal ultrasound needs further study.

Keywords: Cholangiocarcinoma, Epidemiology, Diagnosis, Therapy, Palliation, Systematic review