Background: Cholangiocarcinoma (CCA) is the second most frequent primary liver tumor and defined as the heterogeneous group of tumors derived from cells in the biliary tree.
Methods and results: Based on the anatomical locations (intrahepatic, perihilar, and distal), there are various approaches to the diagnosis and treatment of CCA. Imaging modalities, staging classifications, understandings around natural behavior of CCA, and therapeutic strategies have had remarkable progress in recent years.
Conclusions: This article reviews and discusses the epidemiology, clinical presentation, diagnosis, and treatment modalities of CCA; determines the appropriate inclusion and exclusion criteria for liver transplantation (LT); and defines the risk of disease progression for patients in the waiting list of LT.
Keywords: Cholangiocarcinoma; Hepatectomy; Liver surgery; Liver transplantation; Neoadjuvant therapy.