Intrahepatic Cholangiocarcinoma

Intrahepatic cholangiocarcinoma is an aggressive malignancy. For patients who present with localized disease, surgical resection remains the only potentially curative treatment. Similar to the treatment of other liver malignancies, the principle surgical approach for iCCA should be a margin-negative hepatic resection with preservation of a liver remnant of adequate size and function. Regional lymphadenectomy is recommended at the time of hepatectomy due to the importance of nodal involvement on staging and prognosis. Given the substantial recurrence rates observed even after curative-intent resection, perioperative systemic therapy may have value, with recent prospective data suggesting adjuvant capecitabine may be the standard therapy recommended for most patients. For those with metastatic or unresectable disease, systemic chemotherapy and locoregional modalities are recommended. In the future, improved understanding of the genetic and molecular underpinnings of iCCA tumorigenesis will lead to improved targeted therapies and better outcomes for these patients. This review contains 4 figures, 1 table and 35 references. Key Words: biliary tract cancer, chemotherapy, hepatectomy, intrahepatic cholangiocarcinoma, Klatskin, liver cancer, liver resection, lymphadenectomy, vascular resection