Intrahepatic cholangiocarcinoma (iCCA) is a rare malignancy arising from biliary tract epithelium within bile ducts proximal to the secondary biliary radicles. The majority of patients are diagnosed with locally advanced or metastatic disease at presentation. Surgical resection remains the only potentially curative option, but poses unique challenges due to the large size and aggressive behavior of these tumors. Areas covered: The goal of surgical management of iCCA is margin negative (R0) hepatic resection with preservation of adequate size liver remnant and function. Data regarding role of staging laparoscopy, margin status, portal lymphadenectomy, and vascular resection for iCCA are reviewed. Perioperative systemic therapy may have value, although prospective data have been lacking. Recurrence rates remain high even after R0 resection; among patients with recurrent disease limited to the liver, re-resection or locoregional therapies may play a role. Liver transplantation may be an option for select patients with very early-stage iCCA, although this should be done on a protocol-only basis. Expert commentary: Appropriate preoperative patient selection and surgical technique are paramount to ensure optimal oncologic outcomes for patients with resectable iCCA. Improving systemic and locoregional therapy options may help decrease recurrence rates and improve long-term survival for this aggressive malignancy.
Keywords: Cholangiocarcinoma; bile duct; biliary tract cancer; intrahepatic; resection; surgery.