• Gemma Gatta, Riccardo Capocaccia, Laura Botta, Sandra Mallone, Roberta De Angelis, Eva Ardanaz, Harry Comber, Nadya Dimitrova, Maarit K Leinonen, Sabine Siesling, Jan M van der Zwan, Liesbet Van Eycken, Otto Visser, Maja P Žakelj, Lesley A Anderson, Francesca Bella, Innos Kaire, Renée Otter, Charles A Stiller, Annalisa Trama, and RARECAREnet working group.
• Evaluative Epidemiology Unit, Fondazione IRCCS, Istituto Nazionale dei Tumori, Milan, Italy. Electronic address: gemma.gatta@istitutotumori.mi.it.
• Lancet Oncol. 2017 Aug 1; 18 (8): 1022-1039.

BackgroundRare 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.MethodsWe 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.FindingsRare 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.InterpretationOur 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.FundingThe European Commission (Chafea).Copyright © 2017 Elsevier Ltd. All rights reserved.

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