Abstract
Intrahepatic cholangiocarcinoma (iCCA) is a rare and complex malignancy of the biliary epithelium. Due to its silent presentation, patients are frequently diagnosed late in their disease course, resulting in poor overall survival. Advances in molecular profiling and targeted therapies have improved medical management, but long-term survival is rarely seen with medical therapy alone. Surgical resection offers a survival advantage, but negative oncologic margins are difficult to achieve, recurrence rates are high, and the need for adequate future liver remnant limits the extent of resection. Advances in neoadjuvant and adjuvant treatments have broadened patient treatment options, and these agents are undergoing active investigation, especially in the setting of advanced, initially unresectable disease. For those who are not able to undergo resection, liver transplantation is emerging as a potential curative therapy in certain cases. Patient selection, favorable tumor biology, and a protocolized, multidisciplinary approach are ultimately necessary for best patient outcomes. This review will discuss the current surgical management of locally advanced, liver-limited intrahepatic cholangiocarcinoma as well as the role of liver transplantation for select patients with background liver disease.
Highlights
Intrahepatic cholangiocarcinoma occurs proximal to the segmental biliary ducts and is classically divided into three subtypes based on growth pattern: mass-forming, periductal infiltrating, and intraductal [1,25]
When adjusting for confounders such as tumor differentiation and node-positive disease, results from ESPAC-3, which evaluated chemotherapy versus observation following resection of pancreato-biliary tumors, showed a statistically significant survival benefit for patients who received adjuvant therapy [89]. These data suggest patients would benefit from this adjunct, especially those with high-risk features or positive histologic margins following resection [90]
Additional adjuncts in the neoadjuvant setting for Intrahepatic cholangiocarcinoma (iCCA) include trans-arterial chemoembolization (TACE), drug-eluting bead chemoembolization, trans-arterial radioembolization (TARE), proton beam therapy (PBT), and hepatic artery infusion pumps [94,101,102,103,104,105]
Summary
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Data from the multicenter phase III ABC-02 trial for treatment of advanced biliary tract cancers demonstrate a median overall patient survival of only. Transplantation offers the advantage of the widest margins of resection, treatment of underlying liver disease, and obviates the concern for FLR function [12]. In this era of persistent donor organ shortage, concerns for cancer recurrence, related mortality, and resource allocation have limited the wide application of LT for the treatment of intrahepatic cholangiocarcinoma. We review the current literature on the surgical management of iCCA as well as the emerging role of liver transplantation in the treatment of this aggressive biliary tract malignancy
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