6533b858fe1ef96bd12b6337
RESEARCH PRODUCT
Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study
Fabio FalciniCharles A. StillerDiego SerrainoAntonella Sutera SardoKerri Clough-gorrMario FuscoVéronique BouvierSilvia FrancisciAlain MonnereauCarmen NavarroAlessandro BarchielliAnnalisa TramaBénédicte Lapôtre-ledouxStanislaw GozdzIsabelle KonzelmannJ. BłaszczykLaura BottaSimona BaraFlavio SensiGonçalo Forjaz De LacerdaNadya DimitrovaLucia MangonePamela MinicozziMaria MichiaraNeville CallejaM UsalaE MaraniInnos KaireTom Børge JohannesenLadislav DušekSabine LuttmannM AutelitanoAnne Sophie WoronoffVincent K Y HoVitale FrancescoSilvano PifferJan Maarten Van Der ZwanDyfed Wyn HuwsDavid BrewsterMargit MägiZdravka ValerianovaAnna Luisa CaiazzoMaja Primic ŽAkeljAdriano GiacominE. AlmarF BellaEllen BenhamouArantza Lopez De MunainPatrick ArveuxClaire SchvartzJosé María Díaz GarcíaM. RobaszkiewiczIsabelle BaldiOtto VisserMohsen MousaviMaria José BentoLesley A. AndersonChakameh Safaei DibaMariano SantaquilaniGemma GattaTina ŽAgarPina CandelaJadwiga RachtanMaría José SánchezAndrea BordoniMonika HacklGabriele Schubert-fritschleNea MalilaDiego SalmerónMassimo RuggeCarmen TereanuMaarit LeinonenJem RashbassArturo IannelliMilena SantAntonino Ziino ColaninoMarià CarullaChristine BouchardyKeiu PaapsiMarc ColonnaJon G. JonassonBrigitte TrétarreA.v. GuizardRiccardo CapocacciaXavier TroussardJan HeidrichDominic AgiusKamila KepskaDaniela PierannunzioSanta PildavaStefano FerrettiSilvia RossiGuido MazzoleniRafael Marcos-grageraGiedre SmailyteGianbattista SpagnoliNancy Van DammeRoland StabenowAnna GavinPaolo BailiAnselmo MadedduFabio PannozzoM. SekerijaSilvia EssSandra MalloneRosa FilibertiPascale GrosclaudeFlorence MolinieS MasperoAlice NenneckeRosario TuminoRenée OtterAna Torrella-ramosFabrizio StracciGiovanna TagliabueMichel VeltenAndrea TavillaMarc MaynadiéArnold KnijnConchi Moreno-iribasEva ArdanazM.a. GentiliniPaolo RicciLiesbet Van EyckenAna MirandaSabine SieslingNerea LarrañagaRoberta De AngelisHarry ComberPaolo CollarileK. HenauBernd HolleczekAnne Marie BouvierJutta EngelJaume Galceransubject
Male0301 basic medicinePathologypopulation-based registriesCancer Care Facilities; Delivery of Health Care; Europe; Female; Hospitalization; Humans; Incidence; Male; Neoplasms; Rare Diseases; Registries; Survival Rate; Oncology0302 clinical medicineNeoplasmsMedicineRegistriesmedia_commonTumors -- Treatment -- Europeeducation.field_of_studyRelative survivalIncidenceIncidence (epidemiology)RARECARE projectEuropeHospitalizationSurvival RateOncology030220 oncology & carcinogenesis/dk/atira/pure/sustainabledevelopmentgoals/good_health_and_well_beingFemalemedicine.medical_specialtyHealth surveysPopulationSocio-culturaleCancer Care Facilities03 medical and health sciencesRare DiseasesSDG 3 - Good Health and Well-beingHumansmedia_common.cataloged_instanceCancer -- MortalityRisk factorEuropean unioneducationSurvival rateOncology cancer burden incidence rare cancerpopulation-based registriesrare cancers cancer registry RARECAREbusiness.industryRare cancerCancer -- Patients -- Long-term careCancer registry030104 developmental biologycancer burdenbusinessDelivery of Health CareDemographyRare diseasedescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2017-01-01 |