Perihilar cholangiocarcinoma: Current therapy

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.