Management of Intrahepatic Cholangiocarcinoma: A Narrative Review

Simple Summary Intrahepatic cholangiocarcinoma is a rare primary liver tumor with a poor prognosis and a 5-year survival rate of 9%. While surgical resection offers the best chance of long-term survival, only a subset of patients will present with resectable disease at presentation, and upwards of 70% of patients experience recurrence following resection. Consequently, systemic therapies play an important role in the management of resectable disease. This review summarizes the approach to resectable intrahepatic cholangiocarcinoma and reviews current evidence from clinical trials regarding systemic and targeted therapies.Abstract The management of resectable intrahepatic cholangiocarcinoma remains a challenge due to the high risk of recurrence. Numerous clinical trials have identified effective systemic therapies for advanced biliary tract cancer; however, fewer trials have evaluated systemic therapies in the perioperative period. The objective of this review is to summarize the current recommendations regarding the diagnosis, surgical resection, and systemic therapy for anatomically resectable intrahepatic cholangiocarcinoma. Our review demonstrates that surgical resection with microscopic negative margins and lymphadenectomy remains the cornerstone of treatment. High-level evidence regarding specific systemic therapies for use in resectable intrahepatic cholangiocarcinoma remains sparse, as most of the evidence is extrapolated from trials involving heterogeneous tumor populations. Targeted therapies are an evolving practice for intrahepatic cholangiocarcinoma with most evidence coming from phase II trials. Future research is required to evaluate the use of neoadjuvant therapy for patients with resectable and borderline resectable disease.