UCSF

Key PointsEach anatomical subtype of cholangiocarcinoma, intrahepatic (iCCA), perihilar (pCCA) and distal (dCCA), has a distinct epidemiology, biology, and prognosis, thus necessitating different management approachesFluorescence in situ hybridization (FISH) has improved the diagnostic performance of conventional cytology for the detection of pCCA and dCCA; several emerging diagnostic modalities, including liquid biopsy techniques, might further improve cholangiocarcinoma diagnosisNeoadjuvant chemoradiotherapy followed by liver transplantation offers the best outcomes for a subset of patients with pCCA; liver transplantation might also be an option for patients with very early stage iCCAEmerging evidence indicates that high-dose, conformal external-beam radiation therapy is a potential treatment option for patients with localized, unresectable iCCAAn enhanced understanding of the potential driver genetic aberrations in cholangiocarcinomas has heralded several novel drugs for advanced-stage disease, including FGFR inhibitors and IDH inhibitors; targeted therapy and immunotherapy combinations also hold promise