Burden and centralised treatment in Europe of rare tumours

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 TramaRARECAREnet working group, Rosa Filiberti, Lucia Mangone

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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
JournalThe Lancet Oncology
Volume18
Issue number8
DOIs
Publication statusPublished - Aug 2017

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Neoplasms
Survival
Incidence
Slovenia
Registries
European Union
Netherlands
Tiletamine
Cancer Care Facilities
Bulgaria
Belgium
Case Management
Finland
Ireland
Sarcoma
Spain
Population
Early Diagnosis
Referral and Consultation

Keywords

  • Journal Article

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Burden and centralised treatment in Europe of rare tumours. / Gatta, Gemma; Capocaccia, Riccardo; Botta, Laura; Mallone, Sandra; De Angelis, Roberta; Ardanaz, Eva; Comber, Harry; Dimitrova, Nadya; Leinonen, Maarit K; Siesling, Sabine; van der Zwan, Jan M; Van Eycken, Liesbet; Visser, Otto; Žakelj, Maja P; Anderson, Lesley A; Bella, Francesca; Kaire, Innos; Otter, Renée; Stiller, Charles A; Trama, Annalisa; group, RARECAREnet working; Filiberti, Rosa; Mangone, Lucia.

In: The Lancet Oncology, Vol. 18, No. 8, 08.2017, p. 1022-1039.

Research output: Contribution to journalArticle

Gatta, G, Capocaccia, R, Botta, L, Mallone, S, De Angelis, R, Ardanaz, E, Comber, H, Dimitrova, N, Leinonen, MK, Siesling, S, van der Zwan, JM, Van Eycken, L, Visser, O, Žakelj, MP, Anderson, LA, Bella, F, Kaire, I, Otter, R, Stiller, CA, Trama, A, group, RARECAREW, Filiberti, R & Mangone, L 2017, 'Burden and centralised treatment in Europe of rare tumours', The Lancet Oncology, vol. 18, no. 8, pp. 1022-1039. https://doi.org/10.1016/S1470-2045(17)30445-X
Gatta, Gemma ; Capocaccia, Riccardo ; Botta, Laura ; Mallone, Sandra ; De Angelis, Roberta ; Ardanaz, Eva ; Comber, Harry ; Dimitrova, Nadya ; Leinonen, Maarit K ; Siesling, Sabine ; van der Zwan, Jan M ; Van Eycken, Liesbet ; Visser, Otto ; Žakelj, Maja P ; Anderson, Lesley A ; Bella, Francesca ; Kaire, Innos ; Otter, Renée ; Stiller, Charles A ; Trama, Annalisa ; group, RARECAREnet working ; Filiberti, Rosa ; Mangone, Lucia. / Burden and centralised treatment in Europe of rare tumours. In: The Lancet Oncology. 2017 ; Vol. 18, No. 8. pp. 1022-1039.
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TY - JOUR

T1 - Burden and centralised treatment in Europe of rare tumours

AU - Gatta, Gemma

AU - Capocaccia, Riccardo

AU - Botta, Laura

AU - Mallone, Sandra

AU - De Angelis, Roberta

AU - Ardanaz, Eva

AU - Comber, Harry

AU - Dimitrova, Nadya

AU - Leinonen, Maarit K

AU - Siesling, Sabine

AU - van der Zwan, Jan M

AU - Van Eycken, Liesbet

AU - Visser, Otto

AU - Žakelj, Maja P

AU - Anderson, Lesley A

AU - Bella, Francesca

AU - Kaire, Innos

AU - Otter, Renée

AU - Stiller, Charles A

AU - Trama, Annalisa

AU - group, RARECAREnet working

AU - Filiberti, Rosa

AU - Mangone, Lucia

N1 - Copyright © 2017 Elsevier Ltd. All rights reserved.

PY - 2017/8

Y1 - 2017/8

N2 - 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).

AB - 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).

KW - Journal Article

U2 - 10.1016/S1470-2045(17)30445-X

DO - 10.1016/S1470-2045(17)30445-X

M3 - Article

VL - 18

SP - 1022

EP - 1039

JO - The Lancet Oncology

JF - The Lancet Oncology

SN - 1470-2045

IS - 8

ER -