Latest advances in cholangiocarcinoma

Abstract

Biliary tract cancers include cholangiocarcinoma (CCA) and gallbladder cancer (GBC). Despite being considered rare tumours, their incidence is increasing, especially due to an increased incidence of intrahepatic cholangiocarcinoma (iCCA).1 The majority of patients are diagnosed at late stages, mainly due to challenges at the time of diagnosis.2 This means that for the majority of patients, the only treatment option available is palliative management; thus, prognosis remains poor with a 5-year survival of around 5%-15% when all stages are analysed together.1 This current special issue includes an overview of the main areas of interest in the field of cholangiocarcinoma.3 Firstly, aiming to improve patients’ outcomes, early diagnosis is key. In order to achieve this, in depth knowledge of current endoscopic diagnostic techniques is crucial.4 In addition, the development of new strategies and technologies is the only way of securing adequate early diagnosis and acquisition of tissue to secure sufficient sample for diagnostic and molecular profiling purposes. Endoscopy is also key for the management of one of the most common and clinically relevant complications identified on patients with biliary malignancies: malignant biliary obstruction. Malignant biliary obstruction is considered one of the oncological emergencies and its early release is of much importance to avoid life threatening complications and prevent early and potentially avoidable clinical deterioration. Secondly, treatment for patients with the advanced disease currently relies on the use of palliative chemotherapy.1 However, the emergence of targeted therapies is rapidly changing this,5 especially for iCCA which are most likely to harbour targetable alterations such as fibroblast growth factor receptor (FGFR)-2 fusions and isocitrate dehydrogenase (IDH)-1 mutations, within others. Despite recent studies supporting the role of these precision medicine strategies in cholangiocarcinoma, lots of unanswered questions remain and will need specific research to better understand, not only primary and secondary resistance to these treatments, but also adequate sequencing and combination strategies.6 Thirdly, none of the advances on diagnosis and treatment strategies could have taken place without a close collaboration between basic, translational and clinical researchers. Such collaborations allow identification of diagnostic biomarkers, predictive tools for adequate treatment selection and also understanding of acquired secondary resistance to novel therapies. Within the field of cholangiocarcinoma, many unmet needs can be identified and lots of effort is going into getting this sorted with the aim of moving the field forward.7 In summary, diagnosis and management of cholangiocarcinoma is a rapidly evolving field that requires teamwork and multidisciplinary collaboration to benefit our current and future patients. The articles included in this special issue provide an overview of the current knowledge and unanswered questions for some of the main topics of interest.4, 6, 7 Dr Angela Lamarca received funding from The Christie Charity and the European Union's Horizon 2020 Research and Innovation Programme [grant number 825510, ESCALON]. Travel and educational support from Ipsen, Pfizer, Bayer, AAA, SirtEx, Novartis, Mylan and Delcath; speaker honoraria from Merck, Pfizer, Ipsen, Incyte, AAA, QED and Servier; advisory honoraria from EISAI, Nutricia Ipsen, QED, Roche and Servier; member of the Knowledge Network and NETConnect Initiatives funded by Ipsen. Data sharing is not applicable to this article as no new data were created or analysed in this study.