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Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study

2017; Elsevier BV; Volume: 18; Issue: 8 Linguagem: Inglês

10.1016/s1470-2045(17)30445-x

1474-5488

Gemma Gatta, Riccardo Capocaccia, Laura Botta, Sandra Mallone, Roberta De Angelis, Eva Ardanáz, Harry Comber, Nadya Dimitrova, Maarit K. Leinonen, Sabine Siesling, Jan Maarten van der Zwan, Liesbet Van Eycken, Otto Visser, Maja P Žakelj, Lesley Anderson, Francesca Bella, Innos Kaire, R. Otter, Charles Stiller, Annalisa Trama, Monika Hackl, K. Henau, Nancy Van Damme, Z. Valerianova, Mario Šekerija, Ladislav Dušek, Margit Mägi, Keiu Paapsi, Nea Malila, 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, Guido Mazzoleni, Adriano Giacomin, Antonella Sutera Sardo, Alessandro Barchielli, Diego Serraino, Paolo Collarile, Fabio Pannozzo, 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, Anna Luisa Caiazzo, Arturo Iannelli, Flavio Sensi, Silvano Piffer, M Gentilini, A Madeddu, Antonino Ziino Colanino, S. Maspero, P. Candela, Fabrizio Stracci, Giovanna Tagliabue, Massimo Rugge, Paolo Baili, Pamela Minicozzi, Milena Sant, Carmen Ţereanu, Silvia Francisci, Andrea Tavilla, Daniela Pierannunzio, Silvia Rossi, Mariano Santaquilani, Arnold Knijn, Santa Pildava, Giedrė Smailytė, Neville Calleja, Dominic Agius, Tom Børge Johannesen, Jadwiga Rachtan, Stanisław Góźdż, Jerzy Błaszczyk, Kamila Kępska, G. Forjaz de Lacerda, María José Bento, Ana Miranda, Chakameh Safaei Diba, Tina Žagar, 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 Galcerán, Marià Carulla, Seyed Mohsen Mousavi, Christine Bouchardy, S.M. Ess, Andrea Bordoni, Isabelle Konzelmann, Jem Rashbass, Anna Gavin, David Brewster, Dyfed Wyn Huws, Vincent K.Y. Ho, E Benhamou,

Neuroendocrine Tumor Research Advances

Rare cancers pose challenges for diagnosis, treatments, and clinical decision making. Information about rare cancers is scant. The RARECARE project defined rare cancers as those with an annual incidence of less than six per 100 000 people in European Union (EU). We updated the estimates of the burden of rare cancers in Europe, their time trends in incidence and survival, and provide information about centralisation of treatments in seven European countries.We analysed data from 94 cancer registries for more than 2 million rare cancer diagnoses, to estimate European incidence and survival in 2000-07 and the corresponding time trends during 1995-2007. Incidence was calculated as the number of new cases divided by the corresponding total person-years in the population. 5-year relative survival was calculated by the Ederer-2 method. Seven registries (Belgium, Bulgaria, Finland, Ireland, the Netherlands, Slovenia, and the Navarra region in Spain) provided additional data for hospitals treating about 220 000 cases diagnosed in 2000-07. We also calculated hospital volume admission as the number of treatments provided by each hospital rare cancer group sharing the same referral pattern.Rare cancers accounted for 24% of all cancers diagnosed in the EU during 2000-07. The overall incidence rose annually by 0.5% (99·8% CI 0·3-0·8). 5-year relative survival for all rare cancers was 48·5% (95% CI 48·4 to 48·6), compared with 63·4% (95% CI 63·3 to 63·4) for all common cancers. 5-year relative survival increased (overall 2·9%, 95% CI 2·7 to 3·2), from 1999-2001 to 2007-09, and for most rare cancers, with the largest increases for haematological tumours and sarcomas. The amount of centralisation of rare cancer treatment varied widely between cancers and between countries. The Netherlands and Slovenia had the highest treatment volumes.Our study benefits from the largest pool of population-based registries to estimate incidence and survival of about 200 rare cancers. Incidence trends can be explained by changes in known risk factors, improved diagnosis, and registration problems. Survival could be improved by early diagnosis, new treatments, and improved case management. The centralisation of treatment could be improved in the seven European countries we studied.The European Commission (Chafea).