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

Gemma Gatta*, Riccardo Capocaccia, Laura Botta, Sandra Mallone, Roberta De Angelis, Eva Ardanaz, Harry Comber, Nadya Dimitrova, Maarit K. Leinonen, Sabine Siesling, Jan Maarten van der Zwan, Liesbet Van Eycken, Otto Visser, Maja P. Žakelj, Lesley A. Anderson, Francesca Bella, Innos Kaire, Renée Otter, Charles A. Stiller, Annalisa TramaMonika Hackl, Kris Henau, Nancy Van Damme, Zdravka Valerianova, Mario Sekerija, Ladislav Dušek, Margit Mägi, Keiu Paapsi, Nea Malila, Michel Velten, Xavier Troussard, Veronique Bouvier, Anne Valérie Guizard, Anne Marie Bouvier, Patrick Arveux, Marc Maynadié, Anne Sophie Woronoff, Michel Robaszkiewicz, Isabelle Baldi, Alain Monnereau, Brigitte Tretarre, Marc Colonna, Florence Molinié, Simona Bara, Claire Schvartz, Bénédicte Lapôtre-Ledoux, Pascale Grosclaude, Roland Stabenow, Sabine Luttmann, Alice Nennecke, Jutta Engel, Gabriele SchubertFritschle, Jan Heidrich, Bernd Holleczek, Jón Gunnlaugur Jónasson, Kerri Clough-Gorr, Guido Mazzoleni, Adriano Giacomin, Antonella Sutera Sardo, Alessandro Barchielli, Diego Serraino, Paolo Collarile, Fabio Pannozzo, Paolo Ricci, Mariangela Autelitano, Gianbattista Spagnoli, Mario Fusco, Mario Usala, Francesco Vitale, Maria Michiara, Rosario Tumino, Lucia Mangone, Fabio Falcini, Stefano Ferretti, Rosa Angela Filiberti, Enza Marani, Anna Luisa Caiazzo, Arturo Iannelli, Flavio Sensi, Silvano Piffer, Maria Gentilini, Anselmo Madeddu, Antonino Ziino Colanino, Sergio Maspero, Pina Candela, Fabrizio Stracci, Giovanna Tagliabue, Massimo Rugge, Paolo Baili, Pamela Minicozzi, Milena Sant, Carmen Tereanu, Silvia Francisci, Andrea Tavilla, Daniela Pierannunzio, Silvia Rossi, Mariano Santaquilani, Arnold Knijn, Santa Pildava, Giedre Smailyte, Neville Calleja, Dominic Agius, Tom Børge Johannesen, Jadwiga Rachtan, Stanislaw Gózdz, Jerzy Blaszczyk, Kamila Kepska, Gonçalo Forjaz de Lacerda, Maria José Bento, Ana Miranda, Chakameh Safaei Diba, Tina Žagar, Enrique Almar, Nerea Larrañaga, Arantza Lopez de Munain, Ana Torrella-Ramos, José María Díaz García, Rafael Marcos-Gragera, Maria Josè Sanchez, Carmen Navarro, Diego Salmeron, Conchi Moreno-Iribas, Jaume Galceran, Marià Carulla, Mohsen Mousavi, Christine Bouchardy, Silvia M. Ess, Andrea Bordoni, Isabelle Konzelmann, Jem Rashbass, Anna Gavin, David H. Brewster, Dyfed Wyn Huws, Vincent KY Ho, Ellen Benhamou

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

122 Citations (Scopus)

Abstract

Background 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. Methods 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. Findings 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. Interpretation 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. Funding The European Commission (Chafea).

Original languageEnglish
Pages (from-to)1022-1039
Number of pages18
JournalLancet oncology
Volume18
Issue number8
DOIs
Publication statusPublished - 1 Aug 2017
Externally publishedYes

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