Current Management of Perihilar Cholangiocarcinoma and Future Perspectives

Perihilar cholangiocarcinoma is the most common type of biliary tract cancer and is associated with a high mortality, usually due to late presentation. High-resolution cross-sectional imaging modalities are necessary for diagnosis and preoperative planning. Although surgical resection with negative margins offers the only hope for cure, only a small subset of patients are amenable for surgery at the time of diagnosis. Portal vein embolization and biliary tract decompression are important in some patients prior to surgical resection. Liver transplantation in combination with neoadjuvant therapy has resulted in excellent 5-year recurrence-free survival rates in highly selected patients with inoperable disease. Gemcitabine plus cisplatin constitute the backbone of chemotherapy in patients with inoperable metastatic perihilar cholangiocarcinoma. Recent advances in understanding the molecular pathogenesis of CCA have created a growing interest in identifying novel therapies targeting key molecular pathways. Herein, we provide an overview of the most current principles of management of patients with perihilar cholangiocarcinoma.