Abstract

Intrahepatic cholangiocarcinoma (ICC) is a rare, aggressive cancer of the biliary tract. It often presents with locally advanced or metastatic disease, but for patients with early-stage disease, surgical resection with negative margins and portahepatis lymphadenectomy is the standard of care. Recent advancements in ICC include refinement of staging, improvement in liver-directed therapies, clarification of the role of adjuvant therapy based on new randomized controlled trials, and advances in minimally invasive liver surgery. In addition, improvements in neoadjuvant strategies and surgical techniques have enabled expanded surgical indications and reduced surgical morbidity and mortality. However, recurrence rates remain high and more effective systemic therapies are still necessary to improve recurrence-free and overall survival. In this review, we focus on current and emerging surgical principals for the management of ICC including preoperative evaluation, current indications for surgery, strategies for future liver remnant augmentation, technical principles, and the role of neoadjuvant and adjuvant therapies.

Highlights

  • Intrahepatic cholangiocarcinoma (ICC) is a rare aggressive cancer of the biliary tract

  • A large international study of patients who underwent resection of ICC noted that large tumor size, higher number of tumors, microvascular invasion, N1 or NX disease, suspicious/metastatic lymph nodes on preoperative imaging and R1 resection were associated with a higher likelihood of very early recurrence (≤6 months), suggesting some patients may have benefited from neoadjuvant chemotherapy [34]

  • An international multi-institutional study to identify factors associated with adverse prognosis after resection of ICC noted that positive margin status was one of the strongest factors associated with worse overall survival (OS), in addition to multiple lesions (HR, 1.80; p = 0.001) and vascular invasion (HR, 1.59; p = 0.015) [35]

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Summary

Introduction

Intrahepatic cholangiocarcinoma (ICC) is a rare aggressive cancer of the biliary tract. Refinement of liver-directed therapies have expanded treatment options for patients with locally advanced disease and improved local control [6,7,8]. Despite improvements in prognostication and targeted treatment options, overall survival (OS) among patients with ICC remains low with 5-year overall survival of less than 10% [12,13,14]. This is partly related to the fact that the majority of patients with ICC present with either metastatic, or locally advanced, unresectable disease, and effective systemic therapy options are still lacking. We focus on the surgical aspects of managing ICC including current and emerging principles

Preoperative Evaluation
Diagnostic Imaging of ICC
Indications for Surgery
Resection
Lymphadenectomy
Future Liver Remnant Augmentation Strategies
Transplantation for ICC
Neoadjuvant Therapy
Management of Recurrent ICC
Locoregional Therapy for Unresectable ICC
Findings
Conclusions
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