Intrahepatic cholangiocarcinoma: current perspectives

Abstract

Intrahepatic cholangiocarcinoma (ICC) is the second most common malignancy arising from the liver. ICC makes up about 10% of all cholangiocarcinomas. It arises from the peripheral bile ducts within the liver parenchyma, proximal to the secondary biliary radicals. Histologically, the majority of ICCs are adenocarcinomas. Only a minority of patients (15%) present with resectable disease, with a median survival of less than 3 years. Multidisciplinary management of ICC is complicated by large differences in disease course for individual patients both across and within tumor stages. Risk models and nomograms have been developed to more accurately predict survival of individual patients based on clinical parameters. Predictive risk factors are necessary to improve patient selection for systemic treatments. Molecular differences between tumors, such as in the epidermal growth factor receptor status, are promising, but their clinical applicability should be validated. For patients with locally advanced disease, several treatment strategies are being evaluated. Both hepatic arterial infusion chemotherapy with floxuridine and yttrium-90 embolization aim to downstage locally advanced ICC. Selected patients have resectable disease after downstaging, and other patients might benefit because of postponing widespread dissemination and biliary obstruction.

Keywords: developments; diagnosis; intrahepatic cholangiocarcinoma; treatment.

Figure 1

Types of cholangiocarcinoma. Note: Adapted by permission from Macmillan Publishers Ltd: Nat Rev Gastroenterol Hepatol. Blechacz B, Komuta M, Roskams T, Gores GJ. Clinical diagnosis and staging of cholangiocarcinoma. 2011;8(9):512–522. Copyright 2011.

Figure 2

Overall survival in a large cohort of intrahepatic cholangiocarcinoma patients. Note: Reprinted from J Am Coll Surg, 221(2), Doussot A, Groot-Koerkamp B, Wiggers JK, et al., Outcomes after resection of intrahepatic cholangiocarcinoma: external validation and comparison of prognostic models, 452–461, Copyright (2015), with permission from Elsevier. Abbreviation: AJCC, American Joint Committee on Cancer Staging.

Figure 3

Validated intrahepatic cholangiocarcinoma nomogram predicting overall survival. Adapted from Wang et al. Note: Reprinted with permission. © 2013. American Society of Clinical Oncology. All rights reserved. Wang Y, Li J, Xia Y, et al, Prognostic nomogram for intrahepatic cho langiocarcinoma after partial hepatectomy, J Clin Oncol. 31(9):1188–1195. Abbreviations: CEA, carcino-embryonic antigen; LN, lymph node; PI, periductal invasion.