1. Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study
- Author
-
Fabio Falcini, Charles A. Stiller, Diego Serraino, Antonella Sutera Sardo, Kerri Clough-Gorr, Mario Fusco, Véronique Bouvier, Silvia Francisci, Alain Monnereau, Carmen Navarro, Alessandro Barchielli, Annalisa Trama, Bénédicte Lapôtre-Ledoux, Stanislaw Gózdz, Isabelle Konzelmann, J. Błaszczyk, Laura Botta, Simona Bara, Flavio Sensi, Gonçalo Forjaz de Lacerda, Nadya Dimitrova, Lucia Mangone, Pamela Minicozzi, Maria Michiara, Neville Calleja, M Usala, E Marani, Innos Kaire, Tom Børge Johannesen, Ladislav Dušek, Sabine Luttmann, M Autelitano, Anne Sophie Woronoff, Vincent K Y Ho, Francesco Vitale, Silvano Piffer, Jan Maarten van der Zwan, Dyfed Wyn Huws, David H. Brewster, Margit Mägi, Zdravka Valerianova, Anna Luisa Caiazzo, Maja Primic Žakelj, Adriano Giacomin, E. Almar, F Bella, Ellen Benhamou, Arantza Lopez de Munain, Patrick Arveux, Claire Schvartz, José María Díaz García, M. Robaszkiewicz, Isabelle Baldi, Otto Visser, Mohsen Mousavi, Maria José Bento, Lesley A. Anderson, Chakameh Safaei Diba, Mariano Santaquilani, Gemma Gatta, Tina Žagar, Pina Candela, Jadwiga Rachtan, María José Sánchez, Andrea Bordoni, Monika Hackl, Gabriele Schubert-Fritschle, Nea Malila, Diego Salmerón, Massimo Rugge, Carmen Tereanu, Maarit K. Leinonen, Jem Rashbass, Arturo Iannelli, Milena Sant, Antonino Ziino Colanino, Marià Carulla, Christine Bouchardy, Keiu Paapsi, Marc Colonna, Jon G. Jonasson, Brigitte Trétarre, A.V. Guizard, Riccardo Capocaccia, Xavier Troussard, Jan Heidrich, Dominic Agius, Kamila Kepska, Daniela Pierannunzio, Santa Pildava, Stefano Ferretti, Silvia Rossi, Guido Mazzoleni, Rafael Marcos-Gragera, Giedre Smailyte, Gianbattista Spagnoli, Nancy Van Damme, Roland Stabenow, Anna Gavin, Paolo Baili, Anselmo Madeddu, Fabio Pannozzo, M. Sekerija, Silvia Ess, Sandra Mallone, Rosa Filiberti, Pascale Grosclaude, Florence Molinié, S Maspero, Alice Nennecke, Rosario Tumino, Renée Otter, Ana Torrella-Ramos, Fabrizio Stracci, Giovanna Tagliabue, Michel Velten, Andrea Tavilla, Marc Maynadié, Arnold Knijn, Conchi Moreno-Iribas, Eva Ardanaz, M.A. Gentilini, Paolo Ricci, Liesbet Van Eycken, Ana Miranda, Sabine Siesling, Nerea Larrañaga, Roberta De Angelis, Harry Comber, Paolo Collarile, K. Henau, Bernd Holleczek, Anne Marie Bouvier, Jutta Engel, Jaume Galceran, 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., Zakelj 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., Dusek L., Magi M., Paapsi K., Malila N., Velten M., Troussard X., Bouvier V., Guizard A.-V., Bouvier A.-M., Arveux P., Maynadie M., Woronoff A.-S., Robaszkiewicz M., Baldi I., Monnereau A., Tretarre B., Colonna M., Molinie F., Bara S., Schvartz C., Lapotre-Ledoux B., Grosclaude P., Stabenow R., Luttmann S., Nennecke A., Engel J., SchubertFritschle G., Heidrich J., Holleczek B., Jonasson 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., Gozdz S., Blaszczyk J., Kepska K., Lacerda G.F.D., Bento M.J., Miranda A., Diba C.S., Zagar T., Almar E., Larranaga N., Munain A.L.D., Torrella-Ramos A., Garcia 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., and Benhamou E.
- Subjects
Male ,0301 basic medicine ,Pathology ,population-based registries ,Cancer Care Facilities ,Delivery of Health Care ,Europe ,Female ,Hospitalization ,Humans ,Incidence ,Neoplasms ,Rare Diseases ,Registries ,Survival Rate ,Oncology ,0302 clinical medicine ,Medicine ,media_common ,Tumors -- Treatment -- Europe ,education.field_of_study ,Relative survival ,Incidence (epidemiology) ,RARECARE project ,030220 oncology & carcinogenesis ,medicine.medical_specialty ,Health surveys ,Population ,Socio-culturale ,03 medical and health sciences ,SDG 3 - Good Health and Well-being ,media_common.cataloged_instance ,Cancer -- Mortality ,Risk factor ,European union ,education ,Survival rate ,Oncology, cancer burden, incidence, rare cancer,population-based registries ,rare cancers, cancer registry, RARECARE ,business.industry ,Rare cancer ,Cancer -- Patients -- Long-term care ,Cancer registry ,030104 developmental biology ,cancer burden ,business ,Demography ,Rare disease - 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., peer-reviewed
- Published
- 2017
- Full Text
- View/download PDF