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

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; ... (2024). Characteristics and outcome of synchronous bilateral Wilms tumour in the SIOP WT 2001 Study: Report from the SIOP Renal Tumour Study Group (SIOP-RTSG). British journal of cancer, 131(6), pp. 972-981. Nature Publishing Group 10.1038/s41416-024-02799-0

Text s41416-024-02799-0.pdf - Published Version Restricted to registered users only Available under License Publisher holds Copyright. Download (649kB)

BACKGROUND

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.

CLINICAL TRIAL REGISTRATION

NCT00047138.

Interest & Impact

Downloads

0 since deposited on 13 Aug 2024
0 in the past 12 months

Citations

5 Citations in Web of Science ®
6 Citations in Scopus

Search

in Google Scholar™

Services

Actions (login required)

Edit item

Actions (login required)

Edit item