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Lancet Oncol. 2017 Aug;18(8):1022-1039. doi: 10.1016/S1470-2045(17)30445-X. Epub 2017 Jul 4.

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

Author information

Evaluative Epidemiology Unit, Fondazione IRCCS, Istituto Nazionale dei Tumori, Milan, Italy. Electronic address:
Evaluative Epidemiology Unit, Fondazione IRCCS, Istituto Nazionale dei Tumori, Milan, Italy.
Centro Nazionale Prevenzione delle malattie e Promozione della Salute (CNAPPS) Istituto Superiore di Sanità, Rome, Italy.
Dipartimento di Oncologia e Medicina Molecolare, Istituto Superiore di Sanità, Rome, Italy.
CIBER de Epidemiología y Salud Pública (CIBERESP), Spain; Instituto de Salud Pública de Navarra, Pamplona, Spain.
National Cancer Registry Ireland, Cork, Ireland.
Bulgarian National Cancer Registry, Sofia, Bulgaria.
Cancer Society of Finland, Finnish Cancer Registry, Helsinki, Finland.
Department of Research, Netherlands Comprehensive Cancer Organisation (IKNL), Utrecht, the Netherlands.
Belgian Cancer Registry, Brussels, Belgium.
Netherlands Comprehensive Cancer Organisation (IKNL), Utrecht, the Netherlands.
Institute of Oncology Ljubljana, Epidemiology and Cancer Registry, Ljubljana, Slovenia.
Centre for Public Health, School of Medicine, Dentistry and Biomedical Science, Queen's University Belfast, Belfast, UK.
Integrated Cancer Registry of Catania-Messina-Siracusa-Enna, Azienda Ospedaliero-Universitaria Policlinico-Vittorio Emanuale, Catania, Italy.
Department of Epidemiology and Biostatistics, National Institute for Health Development, Tallinn, Estonia.
Belgian Cancer Center, Department of Public Health and Surveillance, Scientific Institute of Public Health, Brussels, Belgium.
National Cancer Registration and Analysis Service, Public Health England, Oxford, UK.

Erratum in



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).

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