Perihilar Cholangiocarcinoma

Hilar cholangiocarcinomas (HCs) are a diverse group of aggressive tumors often diagnosed late in disease due to their typically longitudinal spread along the biliary system. Only approximately one-third of HCs are the candidates for resection, which offers the only chance for cure. However, even following resection, recurrence is common, and 5-year survival rates after surgery remain dismal at 20 to 42%. A thorough preoperative work-up and risk stratification for major surgery are critical to treatment success and maximizing the likelihood of an R0 resection. Due to the relatively few large phase III randomized controlled trials for HC, data are lacking on the optimal adjuvant treatment. Recent results of the BILCAP trial are promising for improved survival after surgery with adjuvant therapy, particularly capecitabine. When resection is not an option, prognosis is poor with median survival of less than 12 months. However, there are multiple chemotherapy-based treatment options that have demonstrated prolonging survival, with combined gemcitabine and cisplatin as first-line therapy. Liver transplantation should be considered on protocol for unresectable HC in the absence of metastatic disease. Palliative options, such as photodynamic therapy or radiation, may also be considered in advanced disease. This review contains 2 figures, 5 tables, and 41 references. Key Words: adjuvant therapy in cholangiocarcinoma, hilar cholangiocarcinoma, Klatskin tumor, metastatic cholangiocarcinoma, perihilar cholangiocarcinoma, portal dissection, surgery for hilar cholangiocarcinoma, outcomes in hilar cholangiocarcinoma