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World J Gastrointest Pathophysiol. Aug 15, 2014; 5(3): 344-354
Published online Aug 15, 2014. doi: 10.4291/wjgp.v5.i3.344
Perihilar cholangiocarcinoma: Current therapy
Wei Zhang, Lu-Nan Yan
Wei Zhang, Lu-Nan Yan, Department of Liver Surgery, Liver Transplantation Division, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
Author contributions: Zhang W and Yan LN contributed equally to this work; Zhang W and Yan LN designed and performed the research; ZW wrote the paper.
Correspondence to: Lu-Nan Yan, MD, PhD, Department of Liver Surgery, Liver Transplantation Division, West China Hospital, Sichuan University, Wuhou District, Chengdu 610041, Sichuan Province, China. yanlunan688@163.com
Telephone: +86-28-81812453 Fax: +86-28-85423724
Received: January 16, 2010
Revised: April 11, 2014
Accepted: June 10, 2014
Published online: August 15, 2014
Processing time: 230 Days and 10.8 Hours
Abstract

Perihilar cholangiocarcinoma, which is a rare primary malignancy, originates from the epithelial cells of the bile duct. Usually invading the periductal tissues and the lymph nodes, perihilar cholangiocarcinoma is commonly diagnosed in the advanced stage of the disease and has a dismal prognosis. Currently, complete hepatectomy is the primary therapy for curing this disease. Perioperative assessment and available surgical procedures can be considered for achieving a negative margin resection, which is associated with long-term survival and better quality of life. For patients with unresectable cholangiocarcinoma, several palliative treatments have been demonstrated to produce a better outcome; and liver transplantation for selected patients with perihilar cholangiocarcinoma is promising and desirable. However, the role of palliative treatments and liver transplantation was controversial and requires more evidence and substantial validity from multiple institutions. In this article, we summarize the data from multiple institutions and discuss the resectability, mortality, morbidity and outcome with different approaches.

Keywords: Cholangiocarcinoma; Klatskin tumor; Surgery; Liver transplantation; Therapy

Core tip: Perihilar cholangiocarcinoma is a type of malignant tumor with vague and insidious symptoms, and is often diagnosed at an advanced stage. Currently, negative margin resection (R0) is the only way to cure patients with perihilar cholangiocarcinoma. In this article, we describe the surgical procedure and the criteria for operation and illustrate the palliative therapy and liver transplantation options for unresectable perihilar cholangiocarcinoma.