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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| Hauptverfasser: | , , , , , , , , , , , , , , , , , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
5 October 2024
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| In: |
British journal of cancer
Year: 2024, Jahrgang: 131, Heft: 6, Pages: 972-981 |
| ISSN: | 1532-1827 |
| DOI: | 10.1038/s41416-024-02799-0 |
| Online-Zugang: | 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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| Verfasserangaben: | 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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| 245 | 1 | 0 | |a Characteristics and outcome of synchronous bilateral Wilms tumour in the SIOP WT 2001 study |b report from the SIOP Renal Tumour Study Group (SIOP-RTSG) |c 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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| 520 | |a 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. | ||
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| 650 | 4 | |a Wilms tumour | |
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| 700 | 1 | |a van den Heuvel-Eibrink, Mary M. |e VerfasserIn |4 aut | |
| 700 | 1 | |a Pritchard-Jones, Katy |e VerfasserIn |4 aut | |
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