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

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发表日期：

2024年7月30日

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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,
Rhoikos Furtwängler

摘要

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