INTRODUCTION : Intrahepatic cholangiocarcinoma (ICC) is an aggressive biliary tract cancer (BTC) with distinct anatomic, molecular, and clinical characteristics that distinguishes it from other BTCs.

METHODS : In this landmark series review, we highlight the critical studies that have defined the surgical management of ICC, as well as several randomized controlled trials that have investigated adjuvant therapy strategies.

RESULTS : Surgical resection is the mainstay of treatment and should involve margin-negative resection with regional lymphadenectomy. Several recently completed and ongoing randomized controlled trials are defining the indications for adjuvant therapy among patients with resected ICC.

CONCLUSIONS : In the near future, improved understanding of the molecular features of ICC should lead to newer targeted therapies as well as a more personalized approach to systemic therapy.