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Characteristics and outcome of synchronous bilateral Wilms tumour in the SIOP WT 2001 study: report from the SIOP Renal Tumour Study Group (SIOP-RTSG)

Among patients with nephroblastoma, those with bilateral disease are a unique population where maximising tumour control must be balanced with preserving renal parenchyma. Methods: The SIOP 2001 protocol recommended surgery after neoadjuvant cycle(s) of Dactinomycin and Vincristine (AV) with respons...

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Main Authors: Sudour-Bonnange, Hélène (Author) , van Tinteren, Harm (Author) , Ramírez-Villar, Gema L. (Author) , Godzinski, Jan (Author) , Irtan, Sabine (Author) , Gessler, Manfred (Author) , Chowdhury, Tanzina (Author) , Audry, Georges (Author) , Fuchs, Joerg (Author) , Powis, Mark (Author) , van de Ven, Cornelis P. (Author) , Okoye, Bruce (Author) , Smeulders, Naima (Author) , Vujanic, Gordan M. (Author) , Verschuur, Arnaud (Author) , L’Herminé-Coulomb, Aurore (Author) , de Camargo, Beatriz (Author) , de Aguirre Neto, Joaquim Caetano (Author) , Schenk, Jens-Peter (Author) , van den Heuvel-Eibrink, Mary M. (Author) , Pritchard-Jones, Katy (Author) , Graf, Norbert (Author) , Bergeron, Christophe (Author) , Furtwängler, Rhoikos (Author)
Format: Article (Journal)
Language:English
Published: 5 October 2024
In: British journal of cancer
Year: 2024, Volume: 131, Issue: 6, Pages: 972-981
ISSN:1532-1827
DOI:10.1038/s41416-024-02799-0
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1038/s41416-024-02799-0
Verlag, lizenzpflichtig, Volltext: https://www.nature.com/articles/s41416-024-02799-0
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Author Notes:Hélène Sudour-Bonnange, Harm van Tinteren, Gema L. Ramírez-Villar, Jan Godzinski, Sabine Irtan, Manfred Gessler, Tanzina Chowdhury, Georges Audry, Joerg Fuchs, Mark Powis, Cornelis P. van de Ven, Bruce Okoye, Naima Smeulders, Gordan M. Vujanic, Arnaud Verschuur, Aurore L’Herminé-Coulomb, Beatriz de Camargo, Joaquim Caetano de Aguirre Neto, Jens Peter Schenk, Mary M. van den Heuvel-Eibrink, Katy Pritchard-Jones, Norbert Graf, Christophe Bergeron and Rhoikos Furtwängler
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Summary:Among patients with nephroblastoma, those with bilateral disease are a unique population where maximising tumour control must be balanced with preserving renal parenchyma. Methods: The SIOP 2001 protocol recommended surgery after neoadjuvant cycle(s) of Dactinomycin and Vincristine (AV) with response-adapted intensification, if needed. Adjuvant treatment was given based on the lesion with the worst histology. Results: Three hundred and twenty seven patients with stage V disease were evaluable: 174 had bilateral Wilms tumour (BWT), 101 unilateral WT and contralateral nephroblastomatosis (NB) and 52 bilateral nephroblastomatosis. In these three groups, the estimated 5y-EFS was 76.1%, 84.6%, and 74.9%, respectively. AV chemotherapy alone was the successful chemotherapy for 58.7% of all the patients and 65.6% of the non-metastatic patients. Among the 174 patients with BWT, 149 (88.2%) had at least one nephron-sparing surgery. Twenty of 61 bilateral stage I patients were treated with four-week AV postoperatively achieving 94.4% 5y-EFS. At last follow-up, 87% of patients had normal renal function. Conclusions: This study demonstrates that AV without anthracyclines is sufficient to achieve NSS and good survival in the majority of patients. For patients with bilateral stage I WT and intermediate risk histology, only four weeks adjuvant AV seems to be sufficient.
Item Description:Online veröffentlicht: 30. Juli 2024
Gesehen am 13.10.2025
Physical Description:Online Resource
ISSN:1532-1827
DOI:10.1038/s41416-024-02799-0

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