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Cholangiocarcinoma 2020: the Next Horizon in Mechanisms and Management

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Cholangiocarcinoma 2020: the Next Horizon in Mechanisms and Management CONSENSUS STATEMENT Cholangiocarcinoma 2020: the next horizon in mechanisms and management Jesus M. Banales 1,2,3 ✉ , Jose J. G. Marin 2,4, Angela Lamarca 5,6, Pedro M. Rodrigues 1, Shahid A. Khan7, Lewis R. Roberts 8, Vincenzo Cardinale9, Guido Carpino 10, Jesper B. Andersen 11, Chiara Braconi 12, Diego F. Calvisi13, Maria J. Perugorria1,2, Luca Fabris 14,15, Luke Boulter 16, Rocio I. R. Macias 2,4, Eugenio Gaudio17, Domenico Alvaro18, Sergio A. Gradilone19, Mario Strazzabosco 14,15, Marco Marzioni20, Cédric Coulouarn21, Laura Fouassier 22, Chiara Raggi23, Pietro Invernizzi 24, Joachim C. Mertens25, Anja Moncsek25, Sumera Rizvi8, Julie Heimbach26, Bas Groot Koerkamp 27, Jordi Bruix2,28, Alejandro Forner 2,28, John Bridgewater 29, Juan W. Valle 5,6 and Gregory J. Gores 8 Abstract | Cholangiocarcinoma (CCA) includes a cluster of highly heterogeneous biliary malignant tumours that can arise at any point of the biliary tree. Their incidence is increasing globally, currently accounting for ~15% of all primary liver cancers and ~3% of gastrointestinal malignancies. The silent presentation of these tumours combined with their highly aggressive nature and refractoriness to chemotherapy contribute to their alarming mortality, representing ~2% of all cancer-related deaths worldwide yearly. The current diagnosis of CCA by non-invasive approaches is not accurate enough, and histological confirmation is necessary. Furthermore, the high heterogeneity of CCAs at the genomic, epigenetic and molecular levels severely compromises the efficacy of the available therapies. In the past decade, increasing efforts have been made to understand the complexity of these tumours and to develop new diagnostic tools and therapies that might help to improve patient outcomes. In this expert Consensus Statement, which is endorsed by the European Network for the Study of Cholangiocarcinoma, we aim to summarize and critically discuss the latest advances in CCA, mostly focusing on classification, cells of origin, genetic and epigenetic abnormalities, molecular alterations, biomarker discovery and treatments. Furthermore, the horizon of CCA for the next decade from 2020 onwards is highlighted. Cholangiocarcinoma (CCA) constitutes a diverse group (20–30%) and iCCA (10–20%)1,6,7. CCA is the second of malignancies emerging in the biliary tree. CCAs are most common primary hepatic malignancy after hepa- divided into three subtypes depending on their anatom- tocellular carcinoma (HCC), comprising approximately ical site of origin: intrahepatic (iCCA), perihilar (pCCA) 15% of all primary liver tumours and 3% of gastrointes- and distal (dCCA) CCA1,2 (Fig. 1). Of note, considered tinal cancers1,6,7. CCAs are usually asymptomatic in early as an independent entity, mixed HCC–CCA tumours stages and, therefore, often diagnosed when the disease are a rare type of liver malignancy sharing features of is already in advanced stages, which highly compromises both iCCA and HCC and presenting an aggressive dis- therapeutic options, resulting in a dismal prognosis1,8. ease course and poor prognosis3,4. iCCAs arise above the CCA is a rare cancer, but its incidence (0.3–6 per 100,000 second- order bile ducts, whereas the point of anatom- inhabitants per year)1 and mortality (1–6 per 100,000 ical distinction between pCCA and dCCA is the inser- inhabitants per year, globally9, not taking into account ✉e- mail: jesus.banales@ tion of the cystic duct. pCCA and dCCA can also be specific regions with incidence >6 per 100,000 habitants 5 biodonostia.org collectively referred to as ‘extrahepatic’ (eCCA) . In the such as South Korea, China and Thailand) have been https://doi.org/10.1038/ USA, pCCA is the single largest group, accounting for increasing in the past few decades worldwide, repre- s41575-020-0310- z approximately 50–60% of all CCAs, followed by dCCA senting a global health problem. Despite advances in NATURE REVIEWS | GASTROENTEROLOGY & HEPATOLOGY VOLUME 17 | SEPTEMBER 2020 | 557 CONSENSUS STATEMENT CCA awareness, knowledge, diagnosis and therapies, and what is envisaged on the horizon for CCA, focus- patient prognosis has not improved substantially in the ing on epidemiology, risk factors, clinical presentation, past decade, with 5- year survival (7–20%) and tumour diagnosis, genetic and epigenetic landscape, molecular recurrence rates after resection still disappointing10–17. perturbations, chemoresistance and therapies. Therefore, a detailed study of these types of cancers is urgently needed to improve patient welfare and out- Methods comes. Considering the high heterogeneity of CCAs, This international group of multidisciplinary experts in individual characterization of these tumours at the CCA (that is, oncologists, surgeons, hepatologists, genet- genomic, epigenetic and molecular levels is an indispen- icists, immunologists, basic scientists) has been inten- sable approach to ascertain their pathogenesis, paving sively collaborating within the ENS- CCA since 2015 the path for new therapeutic options and personalized with the main aims of improving our understanding of medicine. In this expert Consensus Statement, which CCA and the management of patients. In this regard, is endorsed by the European Network for the Study of this expert consensus is endorsed by the ENS- CCA. Cholangiocarcinoma (ENS- CCA), we provide a com- The overall goal of this multidisciplinary statement is prehensive and critical overview of current knowledge to provide a detailed critical overview of the current knowledge in this field, proposing some expert recom- Author addresses mendations and highlighting what is envisaged for the 1Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research next decade. Institute – Donostia University Hospital, University of the Basque Country (UPV/EHU), J.M.B. and G.J.G. identified the areas of interest, San Sebastian, Spain. stratified the consensus statement into the sections pre- 2National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd, sented in the document and assigned them to selected “Instituto de Salud Carlos III”), San Sebastian, Spain. ENS- CCA members or non- European collaborators 3Ikerbasque, Basque Foundation for Science, Bilbao, Spain. (L.R.R., S.G., S.R., J.H. and G.J.G.) who are expert in 4 Experimental Hepatology and Drug Targeting (HEVEFARM), IBSAL, University of each field of knowledge and research. To write this docu- Salamanca, Salamanca, Spain. ment, a PubMed search was conducted by combining 5Department of Medical Oncology, The Christie NHS Foundation Trust, Manchester, UK. the term ‘cholangiocarcinoma’ with the following terms: 6Division of Cancer Sciences, University of Manchester, Manchester, UK. 7Department of Surgery and Cancer, Imperial College London, Hammersmith Hospital, ‘epidemiology’, ‘risk factors’, ‘classification’, ‘cells of ori- London, UK. gin’, ‘diagnosis’, ‘staging’, ‘genetics’, ‘epigenetics’, ‘signal- 8Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine and ling pathways’, ‘epithelial- to- mesenchymal transition’, Science, Rochester, MN, USA. ‘cancer stem cells’, ‘tumour microenvironment’, ‘immuno- 9Department of Medico- Surgical Sciences and Biotechnologies, Sapienza University biology’, ‘in vitro and in vivo models’, ‘biomarkers’, of Rome, Rome, Italy. ‘surgery’, ‘liver transplantation’, ‘therapies’, ‘clinical trials’ 10Department of Movement, Human and Health Sciences, Division of Health Sciences, and ‘chemoresistance’. No specific search dates were University of Rome “Foro Italico”, Rome, Italy. used. All the sections were merged into a first draft by 11 Biotech Research and Innovation Centre (BRIC), Department of Health and Medical P.M.R and J.M.B. and then extensively revised to create Sciences, University of Copenhagen, Copenhagen, Denmark. the final document that was later circulated among 12Institute of Cancer Sciences, University of Glasgow, Glasgow, UK. 13Institute of Pathology, University of Regensburg, Regensburg, Germany. all the authors for further correction, improvement, dis- 14Department of Molecular Medicine, University of Padua School of Medicine, cussion and approval. The data presented in Fig. 2 were Padua, Italy. obtained by combining the values of mortality rates in 15Digestive Disease Section, Yale University School of Medicine, New Haven, CT, USA. men and women for both iCCA and eCCA reported 16MRC- Human Genetics Unit, Institute of Genetics and Molecular Medicine, University in 2019 by Bertuccio et al.9. For the recommendations of Edinburgh, Edinburgh, UK. on CCA management and research priorities, ideas were 17Division of Human Anatomy, Department of Anatomical, Histological, Forensic proposed, discussed and approved after final revision by Medicine and Orthopedics Sciences, Sapienza University of Rome, Rome, Italy. all the authors to reach a consensus. 18Department of Medicine and Medical Specialties, Sapienza University of Rome, Rome, Italy. Epidemiology and risk factors 19The Hormel Institute, University of Minnesota, Austin, MN, USA. 20Clinic of Gastroenterology and Hepatology, Universita Politecnica delle Marche, The global mortality for CCA increased worldwide Ancona, Italy. during the periods 2000–2004, 2005–2009 and 2010– 21INSERM, Université de Rennes, Rennes, France. 2014 (Fig. 2), according to the WHO and Pan American 22Sorbonne Université, INSERM, Centre de Recherche Saint-Antoine (CRSA), Health Organization databases for 32 selected locations Paris, France. in Europe, America, Asia and Oceania9. Furthermore,
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