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Outcome of patients with stage IV high-risk Wilms tumour treated according to the SIOP2001 protocol: A report of the SIOP Renal Tumour Study Group.

dc.contributor.authorPasqualini, Claudia
dc.contributor.authorFurtwangler, Rhoikos
dc.contributor.authorvan-Tinteren, Harm
dc.contributor.authorTeixeira, Roberto A P
dc.contributor.authorAcha, Tomas
dc.contributor.authorHowell, Lisa
dc.contributor.authorVujanic, Gordan
dc.contributor.authorGodzinski, Jan
dc.contributor.authorMelchior, Patrick
dc.contributor.authorSmets, Anne M
dc.contributor.authorCoulomb-L'Hermine, Aurore
dc.contributor.authorBrisse, Herve
dc.contributor.authorPritchard-Jones, Kathy
dc.contributor.authorBergeron, Christophe
dc.contributor.authorde-Camargo, Beatriz
dc.contributor.authorvan-den-Heuvel-Eibrink, Marry M
dc.contributor.authorGraf, Norbert
dc.contributor.authorVerschuur, Arnauld C
dc.contributor.funderGerman CancerAid
dc.contributor.funderSpanish Association Against Cancer (AECC)
dc.contributor.funderCancer Research UK
dc.date.accessioned2023-02-08T14:42:21Z
dc.date.available2023-02-08T14:42:21Z
dc.date.issued2020-03-05
dc.description.abstractHigh-risk (HR) metastatic (stage IV) Wilms tumours (WTs) have a particular poor outcome. Here, we report the results of HR (diffuse anaplastic [DA] or blastemal type [BT]) stage IV WT treated patients according to the HR arm in the SIOP2001 prospective study. From January 2002 to August 2014, 3559 patients with WT were included in the SIOP2001 trial. Among the 525 patients (15%) with metastatic WT, 74 (14%) had stage IV HR-WT. The median age at diagnosis was 5.5 years (range: 1.4-18.3). Thirty-four patients (47%) had BT-WT and 40 (53%) had DA-WT. Five-year event-free survival rates were 44 ± 17% and 28 ± 15% for BT-WT and DA-WT, respectively (p = 0.09). Five-year overall survival rates were 53 ± 17% and 29 ± 16% for BT-WT and DA-WT, respectively (p = 0.03). Metastatic complete response after preoperative treatment was significantly associated with outcome in univariate and multivariate analyses (hazards ratio = 0.3; p = 0.01). Postoperative radiotherapy of metastatic sites might also be beneficial. Forty-three of 74 patients experienced a relapse or progression predominantly in the lungs (80%). The median time to relapse/progression after diagnosis was 7.3 months (range: 1.6-33.3) and 4.9 months (range: 0.7-28.4) for BT-WT and DA-WT, respectively (p = 0.67). This is the first prospective evidence of inferior survival of stage IV BT-WT as compared with historical intermediate-risk WT. Survival of patients with stage IV DA-WT has not improved compared to the previous SIOP93-01 study. These results call for new treatment approaches for patients with HR stage IV WT.
dc.description.sponsorshipThis work was partly funded by the German CancerAid (Deutsche Krebshilfe, grant No: 50-2709-GR2), Association Léon Berard Enfant Cancéreux and Enfant et Santé (Albec), The DaDa Society (Nieuwerkerk a/d Ijssel, The Netherlands), The Paediatric Oncology Centre Society for Research (KOCR, Rotterdam, the Netherlands), Grupo Cooperativo Brasileiro para o Tratamento do Tumour de Wilms (GCBTTW), Spanish Association Against Cancer (AECC), Cancer Research UK (grant refs: C1010/A2889 & C1188/A8687), and National Cancer Research Network support to UK centres for data management, as well as Elterninitiative Krebskranker Kinder im Saarland (Germany) for continuous data management of GPOH centres.
dc.description.versionSi
dc.identifier.citationPasqualini C, Furtwängler R, van Tinteren H, Teixeira RAP, Acha T, Howell L, et al. Outcome of patients with stage IV high-risk Wilms tumour treated according to the SIOP2001 protocol: A report of the SIOP Renal Tumour Study Group. Eur J Cancer. 2020 Mar;128:38-46
dc.identifier.doi10.1016/j.ejca.2020.01.001
dc.identifier.essn1879-0852
dc.identifier.pmid32109849
dc.identifier.unpaywallURLhttps://discovery.ucl.ac.uk/10093818/16/Pritchard-Jones_Outcome%20of%20patients%20with%20stage%20IV%20high-risk%20Wilms%20tumour%20treated%20according%20to%20the%20SIOP2001%20protocol_AAM2.pdf
dc.identifier.urihttp://hdl.handle.net/10668/15181
dc.journal.titleEuropean journal of cancer (Oxford, England : 1990)
dc.journal.titleabbreviationEur J Cancer
dc.language.isoen
dc.organizationHospital Universitario Regional de Málaga
dc.page.number38-46
dc.provenanceRealizada la curación de contenido 30/04/2025
dc.publisherElsevier
dc.pubmedtypeClinical Trial
dc.pubmedtypeJournal Article
dc.pubmedtypeResearch Support, Non-U.S. Gov't
dc.relation.projectID50-2709-GR2
dc.relation.projectIDC1010/A2889
dc.relation.projectIDC1188/A8687
dc.relation.publisherversionhttps://linkinghub.elsevier.com/retrieve/pii/S0959-8049(20)30002-2
dc.rights.accessRightsRestricted Access
dc.subjectAnaplasia
dc.subjectBlastema
dc.subjectCancer
dc.subjectChild
dc.subjectTP53
dc.subjectWilms
dc.subject.decsRecurrencia
dc.subject.decsAnálisis multivariante
dc.subject.decsRadioterapia
dc.subject.decsInforme de investigación
dc.subject.decsTasa de supervivencia
dc.subject.decsSupervivencia sin progresión
dc.subject.meshAdolescent
dc.subject.meshAntineoplastic Combined Chemotherapy Protocols
dc.subject.meshChild
dc.subject.meshChild, Preschool
dc.subject.meshDactinomycin
dc.subject.meshDisease Progression
dc.subject.meshDisease-Free Survival
dc.subject.meshFemale
dc.subject.meshHumans
dc.subject.meshInfant
dc.subject.meshKidney Neoplasms
dc.subject.meshMale
dc.subject.meshNeoplasm Recurrence, Local
dc.subject.meshNeoplasm Staging
dc.subject.meshProspective Studies
dc.subject.meshSurvival Rate
dc.subject.meshTime Factors
dc.subject.meshVincristine
dc.subject.meshWilms Tumor
dc.titleOutcome of patients with stage IV high-risk Wilms tumour treated according to the SIOP2001 protocol: A report of the SIOP Renal Tumour Study Group.
dc.typeresearch article
dc.type.hasVersionSMUR
dc.volume.number128
dspace.entity.typePublication

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