Abstract

Simple SummaryThis review discusses multimodality treatment strategies for intrahepatic cholangiocarcinoma (iCC). Surgical resection remains the only potentially curative therapeutic option and the central cornerstone of treatment. Adjuvant systemic treatment will be recommended after resection or in the palliative setting. Increasing knowledge of phenotypic subclassification and molecular profiling allows investigation of targeted therapies as (neo-)adjuvant treatment. High-dose brachytherapy, internal radiation therapy, and transarterial chemoembolization are among the interventional treatment options being evaluated for unresectable iCC. Given the multiple options of multidisciplinary management, any treatment strategy should be discussed in a multidisciplinary tumor board and treatment should be directed by a specialized treatment center.Intrahepatic cholangiocarcinoma (iCC) is distinguished as an entity from perihilar and distal cholangiocarcinoma and gallbladder carcinoma. Recently, molecular profiling and histopathological features have allowed further classification. Due to the frequent delay in diagnosis, the prognosis for iCC remains poor despite major technical advances and multimodal therapeutic approaches. Liver resection represents the therapeutic backbone and only curative treatment option, with the functional residual capacity of the liver and oncologic radicality being deciding factors for postoperative and long-term oncological outcome. Furthermore, in selected cases and depending on national guidelines, liver transplantation may be a therapeutic option. Given the often advanced tumor stage at diagnosis or the potential for postoperative recurrence, locoregional therapies have become increasingly important. These strategies range from radiofrequency ablation to transarterial chemoembolization to selective internal radiation therapy and can be used in combination with liver resection. In addition, adjuvant and neoadjuvant chemotherapies as well as targeted therapies and immunotherapies based on molecular profiles can be applied. This review discusses multimodal treatment strategies for iCC and their differential use.

Highlights

  • Intrahepatic cholangiocarcinoma represents a malignant entity parting from epithelium cells of the intrahepatic bile ducts proximal to the ductus hepaticus dexter, respectively sinister

  • In this paper we report about more than 600 minimally invasive liver resections (25% Intrahepatic cholangiocarcinoma (iCC)) and found a 90-day mortality of less than

  • The available therapies for iCC are subject to constant innovative change

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Summary

A Multidisciplinary Approach

Felix Krenzien 1,2,† , Nora Nevermann 1, *,† , Alina Krombholz 1 , Christian Benzing 1 , Philipp Haber 1 , Uli Fehrenbach 3 , Georg Lurje 1 , Uwe Pelzer 4 , Johann Pratschke 1 , Moritz Schmelzle 1 and Wenzel Schöning 1. Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Simple Summary: This review discusses multimodality treatment strategies for intrahepatic cholangiocarcinoma (iCC). Surgical resection remains the only potentially curative therapeutic option and the central cornerstone of treatment. Adjuvant systemic treatment will be recommended after resection or in the palliative setting. Increasing knowledge of phenotypic subclassification and molecular profiling allows investigation of targeted therapies as (neo-)adjuvant treatment. High-dose brachytherapy, internal radiation therapy, and transarterial chemoembolization are among the interventional treatment options being evaluated for unresectable iCC. Given the multiple options of multidisciplinary management, any treatment strategy should be discussed in a multidisciplinary tumor board and treatment should be directed by a specialized treatment center

Introduction
Diagnostics
Liver Resection
Results
Role of Lymphadenectomy
Role of Resection Margins
Minimally Invasive Liver Resection
Management of the Future Liver Remnant
Liver Transplantation
Role for Neoadjuvant Chemotherapy in Intrahepatic Cholangiocarcinomas
Adjuvant Chemotherapy
Targeted Therapies
Role of Immunotherapies
Conclusions

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