Looking Forward: Surgical Resection, Liver Transplantation, and Hilar Cholangiocarcinoma

This is a review of recent clinical advances impacting the management of hilar cholangiocarcinoma.

Hilar cholangiocarcinoma (CCA) is the second most common primary liver malignancy, with an incidence in the USA of approximately 1.2 in 100,000. At present, there are no effective medical therapies, and thus far, the only potentially curative options include surgical resection or neoadjuvant chemoradiotherapy followed by liver transplantation. Although surgical resection remains the standard of care, the majority of patients unfortunately present with unresectable disease. Hilar CCA arises in two subsets of patients: those with de novo disease and those with underlying inflammation, which in western countries is typically primary sclerosing cholangitis (PSC). For patients with early-stage unresectable de novo disease and for patients with hilar CCA arising in the setting of PSC, liver transplantation is the only surgical option and can achieve 65% 5-year survival. While surgical resection remains the standard approach for patients with resectable disease, determining resectability continues to be difficult, and careful consideration must be undertaken in this decision process. Recent changes in surgical technique, primarily the use of en bloc resection, have resulted in improved surgical outcomes. Because adjunct medical therapies remain limited, current work has aimed to identify prognostic factors influencing patient outcomes and to improve endoscopic detection of CCA through optimization of biliary fluorescence in situ hybridization (FISH).

At present, surgical resection and liver transplantation continue to be the only effective treatment options for hilar CCA. Recent advances in treating hilar CCA have resulted in refinements of surgical technique, improvements in diagnostic testing, as well as the identification of prognostic factors impacting patient outcomes and survival.