Abstract

Cholangiocarcinomas (CCAs) are a heterogeneous group of rare malignancies originating from the epithelial cells of the biliary tract, which can be classified anatomically as intrahepatic (IHCC), perihilar (PHCC), and distal (DCC) CCA. Although rare, the incidence of CCAs has increased worldwide over the past 3 decades, which may be caused by the following reasons: firstly, diagnosis of CCAs is usually in the 70’s of age, its incidence is inevitably increased with the prosperity of aging society; secondly, more CCAs are detected in recent years because of the development of imagology, especially in developing countries. However, the risk factors of CCAs remain to be confirmed at present. In addition, the 5-year overall survival rate after diagnosis remains low at 10%

Highlights

  • Cholangiocarcinomas (CCAs) are a heterogeneous group of rare malignancies originating from the epithelial cells of the biliary tract, which can be classified anatomically as intrahepatic (IHCC), perihilar (PHCC), and distal (DCC) CCA

  • Surgery is the only curative treatment for CCA patients; less than one-third of patients are resectable at diagnosis as metastasis already occurred [3]

  • The well-known large-scale randomized phase III trial of systemic therapy performed by Valle J et al showed that gemcitabine plus cisplatin was associated with a significant survival advantage without the addition of substantial toxicity, as compared with gemcitabine alone (11.7 months vs 8.1 months) [10]

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Summary

Introduction

Cholangiocarcinomas (CCAs) are a heterogeneous group of rare malignancies originating from the epithelial cells of the biliary tract, which can be classified anatomically as intrahepatic (IHCC), perihilar (PHCC), and distal (DCC) CCA. The 5-year overall survival rate after diagnosis remains low at 10% [1,2]. Surgery is the only curative treatment for CCA patients; less than one-third of patients are resectable at diagnosis as metastasis already occurred [3]. Majority of patients with CCAs develop an early recurrence after resection. The relatively low resection rate and high relapse rate provide the rational of adjuvant strategies to improve prognosis of CCA patients. The highly desmoplastic nature, extensive support by a rich tumor microenvironment, and profound genetic heterogeneity of CCAs all contribute to its therapeutic resistance [4]. Further investigation on the molecular mechanisms of CCA metastasis is eagerly needed to find new diagnostic biomarkers and therapeutic targets

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