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

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

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Published in:British journal of cancer 2024-10, Vol.131 (6), p.972-981
Main Authors: Sudour-Bonnange, Hélène, van Tinteren, Harm, Ramírez-Villar, Gema L., Godzinski, Jan, Irtan, Sabine, Gessler, Manfred, Chowdhury, Tanzina, Audry, Georges, Fuchs, Joerg, Powis, Mark, van de Ven, Cornelis P., Okoye, Bruce, Smeulders, Naima, Vujanic, Gordan M., Verschuur, Arnaud, L’Herminé-Coulomb, Aurore, de Camargo, Beatriz, de Aguirre Neto, Joaquim Caetano, Schenk, Jens Peter, van den Heuvel-Eibrink, Mary M., Pritchard-Jones, Katy, Graf, Norbert, Bergeron, Christophe, Furtwängler, Rhoikos
Format: Article
Language:English
Subjects:
Anthracycline
Chemotherapy
Dactinomycin
Histology
Metastases
Parenchyma
Patients
Renal function
Surgery
Tumors
Vincristine
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Summary:BackgroundAmong patients with nephroblastoma, those with bilateral disease are a unique population where maximising tumour control must be balanced with preserving renal parenchyma.MethodsThe 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.ResultsThree 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.ConclusionsThis 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.Clinical Trial RegistrationNCT00047138
ISSN:0007-0920
1532-1827
1532-1827
DOI:10.1038/s41416-024-02799-0