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Epidemiology of rare cancers and inequalities in oncologic outcomes

2017; Elsevier BV; Volume: 45; Issue: 1 Linguagem: Inglês

10.1016/j.ejso.2017.08.018

1532-2157

Gemma Gatta, Annalisa Trama, Riccardo Capocaccia, Monika Hackl, E. Van Eycken, K. Henau, Nadya Dimitrova, Mario Šekerija, Ladislav Dušek, Margit Mägi, Nea Malila, Maarit K. Leinonen, Michel Velten, Xavier Troussard, Véronique Bouvier, Anne‐Valérie Guizard, Anne–Marie Bouvier, P. Arveux, Marc Maynadié, Anne‐Sophie Woronoff, Michel Robaszkiewicz, Isabelle Baldi, Alain Monnereau, Brigitte Trétarre, Marc Colonna, F. Molinié, Simona Bara, C. Schvartz, Bénédicte Lapôtre-Ledoux, Pascale Grosclaude, Roland Stabenow, Sabine Luttmann, Alice Nennecke, Jutta Engel, G. Schubert-Fritschle, Jan Heidrich, Bernd Holleczek, Jón G. Jónasson, Kerri M. Clough‐Gorr, Harry Comber, Guido Mazzoleni, Adriano Giacomin, Antonella Sutera Sardo, Alessandro Barchielli, Diego Serraino, Roberta De Angelis, Sandra Mallone, Andrea Tavilla, Daniela Pierannunzio, Silvia Rossi, Mariano Santaquilani, Arnold Knijn, Fabio Pannozzo, Valerio Gennaro, Lucia Benfatto, Paolo Ricci, M. Autelitano, Gianbattista Spagnoli, Mario Fusco, M. Usala, Francesco Vitale, Maria Michiara, ­Rosario ­Tumino, Lucìa Mangone, Fabio Falcini, Stefano Ferretti, Rosa Angela Filiberti, Enza Marani, Arturo Iannelli, Flavio Sensi, Silvano Piffer, M Gentilini, A Madeddu, Antonio Ziino, S. Maspero, P. Candela, Fabrizio Stracci, Giovanna Tagliabue, Massimo Rugge, Annalisa Trama, Gemma Gatta, Laura Botta, Riccardo Capocaccia, Santa Pildava, Giedrė Smailytė, Neville Calleja, Tom Børge Johannesen, Jadwiga Rachtan, Stanisław Góźdż, Jerzy Błaszczyk, Kamila Kępska, Forjaz de Lacerda, María José Bento, Ana Miranda, C. Safaei Diba, E. Almar, Nerea Larrañaga, Arantza Lopez de Munain, A. Torrella-Ramos, J.M. Díaz García, Rafael Marcos‐Gragera, Maria Josè Sanchez, Carmen Navarro, Diego Salmerón, Conchi Moreno‐Iribas, Jaume Galceran, Marià Carulla, Seyed Mohsen Mousavi, Christine Bouchardy, Silvia M. Ess, Andrea Bordoni, Isabelle Konzelmann, Jem Rashbass, Anna Gavin, David Brewster, Dyfed Huws, Otto Visser, Magdalena Bielska‐Lasota, Maja Primic-Zakelj, Ian Kunkler, E Benhamou,

Neuroendocrine Tumor Research Advances

Rare cancers epidemiology is better known compared to the other rare diseases. Thanks to the long history of the European population-based cancer registries and to the EUROCARE huge database, the burden of rare cancers has been estimated the European (EU28) population. A considerable fraction of all cancers is represented by rare cancers (24%). They are a heterogeneous group of diseases, but they share similar problems: uncertainty of diagnosis, lack of therapies, poor research opportunities, difficulties in clinical trials, lack of expertise and of centres of reference. This paper analyses the major epidemiological indicators of frequency (incidence and prevalence) and outcome (5-year survival) of all rare cancers combined and of selected rare cancers that will be in depth treated in this monographic issue. Source of the results is the RARECAREnet search tool, a database publicly available. Disparities both in incidence and survival, and consequently in prevalence of rare cancers were reported across European countries. Major differences were shown in outcome: 5-year relative survival for all rare cancers together, adjusted by age and case-mix, varied from 55% or more (Italy, Germany, Belgium and Iceland) and less than 40% (Bulgaria, Lithuania and Slovakia). Similarly, for all the analyzed rare cancers, a large survival gap was observed between the Eastern and the Nordic and Central European regions. Dramatic geographical variations were assessed for curable cancers like testicular and non epithelial ovarian cancers. Geographical difference in the annual age-adjusted incidence rates for all rare cancers together varied between >140 per 100,000 (Italy, Scotland, France, Germany, and Switzerland) and <100 (Finland, Portugal, Malta, and Poland). Prevalence, the major indicator of public health resources needs, was about 7–8 times larger than incidence. Most of rare cancers require complex surgical treatment, thus a multidisciplinary approach is essential and treatment should be provided in centres of expertise and/or in networks including expert centres. Networking is the most appropriate answer to the issues pertaining to rare cancers. Actually, in Europe, an opportunity to improve outcome and reduce disparities is provided by the creation of the European Reference Networks for rare diseases (ERNs). The Joint Action of rare cancers (JARC) is a major European initiative aimed to support the mission of the ERNs. The role of population based cancer registries still remains crucial to describe rare cancers management and outcome in the real word and to evaluate progresses made at the country and at the European level.