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

G. Gatta, R. Capocaccia, L. Botta, S. Mallone, R. De Angelis, E. Ardanaz, H. Comber, N. Dimitrova, M.K. Leinonen, S. Siesling, J.M. van der Zwan, L. Van Eycken, O. Visser, M.P. Žakelj, L.A. Anderson, F. Bella, I. Kaire, R. Otter, C.A. Stiller, A. TramaM. Hackl, K. Henau, N. Van Damme, Z. Valerianova, M. Sekerija, L. Dušek, M. Mägi, K. Paapsi, N. Malila, M. Velten, X. Troussard, V. Bouvier, A.-V. Guizard, A.-M. Bouvier, P. Arveux, M. Maynadié, A.-S. Woronoff, M. Robaszkiewicz, I. Baldi, A. Monnereau, B. Tretarre, M. Colonna, F. Molinié, S. Bara, C. Schvartz, B. Lapôtre-Ledoux, P. Grosclaude, R. Stabenow, S. Luttmann, A. Nennecke, J. Engel, G. SchubertFritschle, J. Heidrich, B. Holleczek, J.G. Jónasson, K. Clough-Gorr, G. Mazzoleni, A. Giacomin, A.S. Sardo, A. Barchielli, D. Serraino, P. Collarile, F. Pannozzo, P. Ricci, M. Autelitano, G. Spagnoli, M. Fusco, M. Usala, F. Vitale, M. Michiara, R. Tumino, L. Mangone, F. Falcini, S. Ferretti, R.A. Filiberti, E. Marani, A.L. Caiazzo, A. Iannelli, F. Sensi, S. Piffer, M. Gentilini, A. Madeddu, A.Z. Colanino, S. Maspero, P. Candela, F. Stracci, G. Tagliabue, M. Rugge, P. Baili, P. Minicozzi, M. Sant, C. Tereanu, S. Francisci, A. Tavilla, D. Pierannunzio, S. Rossi, M. Santaquilani, A. Knijn, S. Pildava, G. Smailyte, N. Calleja, D. Agius, T.B. Johannesen, J. Rachtan, S. Gózdz, J. Blaszczyk, K. Kepska, G.F.D. Lacerda, M.J. Bento, A. Miranda, C.S. Diba, T. Žagar, E. Almar, N. Larrañaga, A.L.D. Munain, A. Torrella-Ramos, J.M.D. García, R. Marcos-Gragera, M.J. Sanchez, C. Navarro, D. Salmeron, C. Moreno-Iribas, J. Galceran, M. Carulla, M. Mousavi, C. Bouchardy, S.M. Ess, A. Bordoni, I. Konzelmann, J. Rashbass, A. Gavin, D.H. Brewster, D.W. Huws, V.K. Ho, E. Benhamou

Research output: Contribution to journalArticle

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). © 2017 Elsevier Ltd
Original languageEnglish
Pages (from-to)1022-1039
Number of pages18
JournalThe Lancet Oncology
Volume18
Issue number8
DOIs
Publication statusPublished - 2017

Fingerprint

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

Keywords

  • antineoplastic agent
  • adult
  • Article
  • Belgium
  • Bulgaria
  • cancer chemotherapy
  • cancer incidence
  • cancer radiotherapy
  • cancer registry
  • cancer surgery
  • cancer survival
  • female
  • Finland
  • hematologic malignancy
  • human
  • Ireland
  • major clinical study
  • male
  • malignant neoplasm
  • Navarre
  • Netherlands
  • population research
  • priority journal
  • rare disease
  • risk factor
  • sarcoma
  • Slovenia
  • survival time
  • treatment planning
  • trend study
  • cancer center
  • Europe
  • health care delivery
  • hospitalization
  • incidence
  • mortality
  • Neoplasms
  • Rare Diseases
  • register
  • statistics and numerical data
  • survival rate
  • Cancer Care Facilities
  • Delivery of Health Care
  • Female
  • Hospitalization
  • Humans
  • Incidence
  • Male
  • Registries
  • Survival Rate

Cite this

Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study. / Gatta, G.; Capocaccia, R.; Botta, L.; Mallone, S.; De Angelis, R.; Ardanaz, E.; Comber, H.; Dimitrova, N.; Leinonen, M.K.; Siesling, S.; van der Zwan, J.M.; Van Eycken, L.; Visser, O.; Žakelj, M.P.; Anderson, L.A.; Bella, F.; Kaire, I.; Otter, R.; Stiller, C.A.; Trama, A.; Hackl, M.; Henau, K.; Van Damme, N.; Valerianova, Z.; Sekerija, M.; Dušek, L.; Mägi, M.; Paapsi, K.; Malila, N.; Velten, M.; Troussard, X.; Bouvier, V.; Guizard, A.-V.; Bouvier, A.-M.; Arveux, P.; Maynadié, M.; Woronoff, A.-S.; Robaszkiewicz, M.; Baldi, I.; Monnereau, A.; Tretarre, B.; Colonna, M.; Molinié, F.; Bara, S.; Schvartz, C.; Lapôtre-Ledoux, B.; Grosclaude, P.; Stabenow, R.; Luttmann, S.; Nennecke, A.; Engel, J.; SchubertFritschle, G.; Heidrich, J.; Holleczek, B.; Jónasson, J.G.; Clough-Gorr, K.; Mazzoleni, G.; Giacomin, A.; Sardo, A.S.; Barchielli, A.; Serraino, D.; Collarile, P.; Pannozzo, F.; Ricci, P.; Autelitano, M.; Spagnoli, G.; Fusco, M.; Usala, M.; Vitale, F.; Michiara, M.; Tumino, R.; Mangone, L.; Falcini, F.; Ferretti, S.; Filiberti, R.A.; Marani, E.; Caiazzo, A.L.; Iannelli, A.; Sensi, F.; Piffer, S.; Gentilini, M.; Madeddu, A.; Colanino, A.Z.; Maspero, S.; Candela, P.; Stracci, F.; Tagliabue, G.; Rugge, M.; Baili, P.; Minicozzi, P.; Sant, M.; Tereanu, C.; Francisci, S.; Tavilla, A.; Pierannunzio, D.; Rossi, S.; Santaquilani, M.; Knijn, A.; Pildava, S.; Smailyte, G.; Calleja, N.; Agius, D.; Johannesen, T.B.; Rachtan, J.; Gózdz, S.; Blaszczyk, J.; Kepska, K.; Lacerda, G.F.D.; Bento, M.J.; Miranda, A.; Diba, C.S.; Žagar, T.; Almar, E.; Larrañaga, N.; Munain, A.L.D.; Torrella-Ramos, A.; García, J.M.D.; Marcos-Gragera, R.; Sanchez, M.J.; Navarro, C.; Salmeron, D.; Moreno-Iribas, C.; Galceran, J.; Carulla, M.; Mousavi, M.; Bouchardy, C.; Ess, S.M.; Bordoni, A.; Konzelmann, I.; Rashbass, J.; Gavin, A.; Brewster, D.H.; Huws, D.W.; Ho, V.K.; Benhamou, E.

In: The Lancet Oncology, Vol. 18, No. 8, 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, Hackl, M, Henau, K, Van Damme, N, Valerianova, Z, Sekerija, M, Dušek, L, Mägi, M, Paapsi, K, Malila, N, Velten, M, Troussard, X, Bouvier, V, Guizard, A-V, Bouvier, A-M, Arveux, P, Maynadié, M, Woronoff, A-S, Robaszkiewicz, M, Baldi, I, Monnereau, A, Tretarre, B, Colonna, M, Molinié, F, Bara, S, Schvartz, C, Lapôtre-Ledoux, B, Grosclaude, P, Stabenow, R, Luttmann, S, Nennecke, A, Engel, J, SchubertFritschle, G, Heidrich, J, Holleczek, B, Jónasson, JG, Clough-Gorr, K, Mazzoleni, G, Giacomin, A, Sardo, AS, Barchielli, A, Serraino, D, Collarile, P, Pannozzo, F, Ricci, P, Autelitano, M, Spagnoli, G, Fusco, M, Usala, M, Vitale, F, Michiara, M, Tumino, R, Mangone, L, Falcini, F, Ferretti, S, Filiberti, RA, Marani, E, Caiazzo, AL, Iannelli, A, Sensi, F, Piffer, S, Gentilini, M, Madeddu, A, Colanino, AZ, Maspero, S, Candela, P, Stracci, F, Tagliabue, G, Rugge, M, Baili, P, Minicozzi, P, Sant, M, Tereanu, C, Francisci, S, Tavilla, A, Pierannunzio, D, Rossi, S, Santaquilani, M, Knijn, A, Pildava, S, Smailyte, G, Calleja, N, Agius, D, Johannesen, TB, Rachtan, J, Gózdz, S, Blaszczyk, J, Kepska, K, Lacerda, GFD, Bento, MJ, Miranda, A, Diba, CS, Žagar, T, Almar, E, Larrañaga, N, Munain, ALD, Torrella-Ramos, A, García, JMD, Marcos-Gragera, R, Sanchez, MJ, Navarro, C, Salmeron, D, Moreno-Iribas, C, Galceran, J, Carulla, M, Mousavi, M, Bouchardy, C, Ess, SM, Bordoni, A, Konzelmann, I, Rashbass, J, Gavin, A, Brewster, DH, Huws, DW, Ho, VK & Benhamou, E 2017, 'Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study', The Lancet Oncology, vol. 18, no. 8, pp. 1022-1039. https://doi.org/10.1016/S1470-2045(17)30445-X
Gatta, G. ; Capocaccia, R. ; Botta, L. ; Mallone, S. ; De Angelis, R. ; Ardanaz, E. ; Comber, H. ; Dimitrova, N. ; Leinonen, M.K. ; Siesling, S. ; van der Zwan, J.M. ; Van Eycken, L. ; Visser, O. ; Žakelj, M.P. ; Anderson, L.A. ; Bella, F. ; Kaire, I. ; Otter, R. ; Stiller, C.A. ; Trama, A. ; Hackl, M. ; Henau, K. ; Van Damme, N. ; Valerianova, Z. ; Sekerija, M. ; Dušek, L. ; Mägi, M. ; Paapsi, K. ; Malila, N. ; Velten, M. ; Troussard, X. ; Bouvier, V. ; Guizard, A.-V. ; Bouvier, A.-M. ; Arveux, P. ; Maynadié, M. ; Woronoff, A.-S. ; Robaszkiewicz, M. ; Baldi, I. ; Monnereau, A. ; Tretarre, B. ; Colonna, M. ; Molinié, F. ; Bara, S. ; Schvartz, C. ; Lapôtre-Ledoux, B. ; Grosclaude, P. ; Stabenow, R. ; Luttmann, S. ; Nennecke, A. ; Engel, J. ; SchubertFritschle, G. ; Heidrich, J. ; Holleczek, B. ; Jónasson, J.G. ; Clough-Gorr, K. ; Mazzoleni, G. ; Giacomin, A. ; Sardo, A.S. ; Barchielli, A. ; Serraino, D. ; Collarile, P. ; Pannozzo, F. ; Ricci, P. ; Autelitano, M. ; Spagnoli, G. ; Fusco, M. ; Usala, M. ; Vitale, F. ; Michiara, M. ; Tumino, R. ; Mangone, L. ; Falcini, F. ; Ferretti, S. ; Filiberti, R.A. ; Marani, E. ; Caiazzo, A.L. ; Iannelli, A. ; Sensi, F. ; Piffer, S. ; Gentilini, M. ; Madeddu, A. ; Colanino, A.Z. ; Maspero, S. ; Candela, P. ; Stracci, F. ; Tagliabue, G. ; Rugge, M. ; Baili, P. ; Minicozzi, P. ; Sant, M. ; Tereanu, C. ; Francisci, S. ; Tavilla, A. ; Pierannunzio, D. ; Rossi, S. ; Santaquilani, M. ; Knijn, A. ; Pildava, S. ; Smailyte, G. ; Calleja, N. ; Agius, D. ; Johannesen, T.B. ; Rachtan, J. ; Gózdz, S. ; Blaszczyk, J. ; Kepska, K. ; Lacerda, G.F.D. ; Bento, M.J. ; Miranda, A. ; Diba, C.S. ; Žagar, T. ; Almar, E. ; Larrañaga, N. ; Munain, A.L.D. ; Torrella-Ramos, A. ; García, J.M.D. ; Marcos-Gragera, R. ; Sanchez, M.J. ; Navarro, C. ; Salmeron, D. ; Moreno-Iribas, C. ; Galceran, J. ; Carulla, M. ; Mousavi, M. ; Bouchardy, C. ; Ess, S.M. ; Bordoni, A. ; Konzelmann, I. ; Rashbass, J. ; Gavin, A. ; Brewster, D.H. ; Huws, D.W. ; Ho, V.K. ; Benhamou, E. / Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study. In: The Lancet Oncology. 2017 ; Vol. 18, No. 8. pp. 1022-1039.
@article{7d29cc03f01b41d79d3b46f62effac78,
title = "Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study",
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). {\circledC} 2017 Elsevier Ltd",
keywords = "antineoplastic agent, adult, Article, Belgium, Bulgaria, cancer chemotherapy, cancer incidence, cancer radiotherapy, cancer registry, cancer surgery, cancer survival, female, Finland, hematologic malignancy, human, Ireland, major clinical study, male, malignant neoplasm, Navarre, Netherlands, population research, priority journal, rare disease, risk factor, sarcoma, Slovenia, survival time, treatment planning, trend study, cancer center, Europe, health care delivery, hospitalization, incidence, mortality, Neoplasms, Rare Diseases, register, statistics and numerical data, survival rate, Cancer Care Facilities, Delivery of Health Care, Female, Hospitalization, Humans, Incidence, Male, Registries, Survival Rate",
author = "G. Gatta and R. Capocaccia and L. Botta and S. Mallone and {De Angelis}, R. and E. Ardanaz and H. Comber and N. Dimitrova and M.K. Leinonen and S. Siesling and {van der Zwan}, J.M. and {Van Eycken}, L. and O. Visser and M.P. Žakelj and L.A. Anderson and F. Bella and I. Kaire and R. Otter and C.A. Stiller and A. Trama and M. Hackl and K. Henau and {Van Damme}, N. and Z. Valerianova and M. Sekerija and L. Dušek and M. M{\"a}gi and K. Paapsi and N. Malila and M. Velten and X. Troussard and V. Bouvier and A.-V. Guizard and A.-M. Bouvier and P. Arveux and M. Maynadi{\'e} and A.-S. Woronoff and M. Robaszkiewicz and I. Baldi and A. Monnereau and B. Tretarre and M. Colonna and F. Molini{\'e} and S. Bara and C. Schvartz and B. Lap{\^o}tre-Ledoux and P. Grosclaude and R. Stabenow and S. Luttmann and A. Nennecke and J. Engel and G. SchubertFritschle and J. Heidrich and B. Holleczek and J.G. J{\'o}nasson and K. Clough-Gorr and G. Mazzoleni and A. Giacomin and A.S. Sardo and A. Barchielli and D. Serraino and P. Collarile and F. Pannozzo and P. Ricci and M. Autelitano and G. Spagnoli and M. Fusco and M. Usala and F. Vitale and M. Michiara and R. Tumino and L. Mangone and F. Falcini and S. Ferretti and R.A. Filiberti and E. Marani and A.L. Caiazzo and A. Iannelli and F. Sensi and S. Piffer and M. Gentilini and A. Madeddu and A.Z. Colanino and S. Maspero and P. Candela and F. Stracci and G. Tagliabue and M. Rugge and P. Baili and P. Minicozzi and M. Sant and C. Tereanu and S. Francisci and A. Tavilla and D. Pierannunzio and S. Rossi and M. Santaquilani and A. Knijn and S. Pildava and G. Smailyte and N. Calleja and D. Agius and T.B. Johannesen and J. Rachtan and S. G{\'o}zdz and J. Blaszczyk and K. Kepska and G.F.D. Lacerda and M.J. Bento and A. Miranda and C.S. Diba and T. Žagar and E. Almar and N. Larra{\~n}aga and A.L.D. Munain and A. Torrella-Ramos and J.M.D. Garc{\'i}a and R. Marcos-Gragera and M.J. Sanchez and C. Navarro and D. Salmeron and C. Moreno-Iribas and J. Galceran and M. Carulla and M. Mousavi and C. Bouchardy and S.M. Ess and A. Bordoni and I. Konzelmann and J. Rashbass and A. Gavin and D.H. Brewster and D.W. Huws and V.K. Ho and E. Benhamou",
note = "Cited By :1 Export Date: 15 February 2018 CODEN: LOANB Correspondence Address: Gatta, G.; Evaluative Epidemiology Unit, Fondazione IRCCS, Istituto Nazionale dei TumoriItaly; email: gemma.gatta@istitutotumori.mi.it",
year = "2017",
doi = "10.1016/S1470-2045(17)30445-X",
language = "English",
volume = "18",
pages = "1022--1039",
journal = "The Lancet Oncology",
issn = "1470-2045",
publisher = "Lancet Publishing Group",
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}

TY - JOUR

T1 - Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study

AU - Gatta, G.

AU - Capocaccia, R.

AU - Botta, L.

AU - Mallone, S.

AU - De Angelis, R.

AU - Ardanaz, E.

AU - Comber, H.

AU - Dimitrova, N.

AU - Leinonen, M.K.

AU - Siesling, S.

AU - van der Zwan, J.M.

AU - Van Eycken, L.

AU - Visser, O.

AU - Žakelj, M.P.

AU - Anderson, L.A.

AU - Bella, F.

AU - Kaire, I.

AU - Otter, R.

AU - Stiller, C.A.

AU - Trama, A.

AU - Hackl, M.

AU - Henau, K.

AU - Van Damme, N.

AU - Valerianova, Z.

AU - Sekerija, M.

AU - Dušek, L.

AU - Mägi, M.

AU - Paapsi, K.

AU - Malila, N.

AU - Velten, M.

AU - Troussard, X.

AU - Bouvier, V.

AU - Guizard, A.-V.

AU - Bouvier, A.-M.

AU - Arveux, P.

AU - Maynadié, M.

AU - Woronoff, A.-S.

AU - Robaszkiewicz, M.

AU - Baldi, I.

AU - Monnereau, A.

AU - Tretarre, B.

AU - Colonna, M.

AU - Molinié, F.

AU - Bara, S.

AU - Schvartz, C.

AU - Lapôtre-Ledoux, B.

AU - Grosclaude, P.

AU - Stabenow, R.

AU - Luttmann, S.

AU - Nennecke, A.

AU - Engel, J.

AU - SchubertFritschle, G.

AU - Heidrich, J.

AU - Holleczek, B.

AU - Jónasson, J.G.

AU - Clough-Gorr, K.

AU - Mazzoleni, G.

AU - Giacomin, A.

AU - Sardo, A.S.

AU - Barchielli, A.

AU - Serraino, D.

AU - Collarile, P.

AU - Pannozzo, F.

AU - Ricci, P.

AU - Autelitano, M.

AU - Spagnoli, G.

AU - Fusco, M.

AU - Usala, M.

AU - Vitale, F.

AU - Michiara, M.

AU - Tumino, R.

AU - Mangone, L.

AU - Falcini, F.

AU - Ferretti, S.

AU - Filiberti, R.A.

AU - Marani, E.

AU - Caiazzo, A.L.

AU - Iannelli, A.

AU - Sensi, F.

AU - Piffer, S.

AU - Gentilini, M.

AU - Madeddu, A.

AU - Colanino, A.Z.

AU - Maspero, S.

AU - Candela, P.

AU - Stracci, F.

AU - Tagliabue, G.

AU - Rugge, M.

AU - Baili, P.

AU - Minicozzi, P.

AU - Sant, M.

AU - Tereanu, C.

AU - Francisci, S.

AU - Tavilla, A.

AU - Pierannunzio, D.

AU - Rossi, S.

AU - Santaquilani, M.

AU - Knijn, A.

AU - Pildava, S.

AU - Smailyte, G.

AU - Calleja, N.

AU - Agius, D.

AU - Johannesen, T.B.

AU - Rachtan, J.

AU - Gózdz, S.

AU - Blaszczyk, J.

AU - Kepska, K.

AU - Lacerda, G.F.D.

AU - Bento, M.J.

AU - Miranda, A.

AU - Diba, C.S.

AU - Žagar, T.

AU - Almar, E.

AU - Larrañaga, N.

AU - Munain, A.L.D.

AU - Torrella-Ramos, A.

AU - García, J.M.D.

AU - Marcos-Gragera, R.

AU - Sanchez, M.J.

AU - Navarro, C.

AU - Salmeron, D.

AU - Moreno-Iribas, C.

AU - Galceran, J.

AU - Carulla, M.

AU - Mousavi, M.

AU - Bouchardy, C.

AU - Ess, S.M.

AU - Bordoni, A.

AU - Konzelmann, I.

AU - Rashbass, J.

AU - Gavin, A.

AU - Brewster, D.H.

AU - Huws, D.W.

AU - Ho, V.K.

AU - Benhamou, E.

N1 - Cited By :1 Export Date: 15 February 2018 CODEN: LOANB Correspondence Address: Gatta, G.; Evaluative Epidemiology Unit, Fondazione IRCCS, Istituto Nazionale dei TumoriItaly; email: gemma.gatta@istitutotumori.mi.it

PY - 2017

Y1 - 2017

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). © 2017 Elsevier Ltd

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). © 2017 Elsevier Ltd

KW - antineoplastic agent

KW - adult

KW - Article

KW - Belgium

KW - Bulgaria

KW - cancer chemotherapy

KW - cancer incidence

KW - cancer radiotherapy

KW - cancer registry

KW - cancer surgery

KW - cancer survival

KW - female

KW - Finland

KW - hematologic malignancy

KW - human

KW - Ireland

KW - major clinical study

KW - male

KW - malignant neoplasm

KW - Navarre

KW - Netherlands

KW - population research

KW - priority journal

KW - rare disease

KW - risk factor

KW - sarcoma

KW - Slovenia

KW - survival time

KW - treatment planning

KW - trend study

KW - cancer center

KW - Europe

KW - health care delivery

KW - hospitalization

KW - incidence

KW - mortality

KW - Neoplasms

KW - Rare Diseases

KW - register

KW - statistics and numerical data

KW - survival rate

KW - Cancer Care Facilities

KW - Delivery of Health Care

KW - Female

KW - Hospitalization

KW - Humans

KW - Incidence

KW - Male

KW - Registries

KW - Survival Rate

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 -