Advances in the management of cholangiocarcinoma

Abstract

Cholangiocarcinoma (CCA) is a primary malignancy of the bile ducts with three anatomically and molecularly distinct entities: Intrahepatic CCA (iCCA), perihilar CCA (pCCA), and distal CCA. As a result of phenotypic and anatomic differences they differ significantly with respect to management. For each type of CCA there have been significant changes in management over the last several years which will be discussed in this review. Although resection remains the standard of care for all types of CCA, liver transplantation has been established as curative treatment for selected patients with pCCA and is being evaluated for iCCA with early success. With respect to systemic therapy capecitabine is now first line adjuvant therapy for all biliary tract malignancies after curative intent resection. Progress in exploiting the pathologic mutations and molecular abnormalities has also yielded regulatory approval of targeted therapy for CCA in patients with acquired alterations in the fibroblast growth factor receptor. There is also increased consensus in managing malignant biliary obstruction associated with CCA where pre-operative biliary stenting is not beneficial while self-expanding metal stents have been shown to be superior to plastic stents in patients who are not surgical candidates.

Keywords: Cholangiocarcinoma; Intrahepatic cholangiocarcinoma; Perihilar cholangiocarcinoma; Liver transplantation; Chemotherapy

Core Tip: This review presents recent advances in the management of cholangiocarcinoma with particular focus on the expanding role for liver transplantation, updated guidelines in the use of chemotherapy, novel applications of individualized therapy targeting the specific mutation profile of tumors, and management of malignant biliary obstruction.