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, H.; van Tinteren, H.; Ramirez-Villar, GL.; Godzinski, J.; Irtan, S.; Gessler, M.; Chowdhury, T.; Audry, G.; Fuchs, J.; Powis, M.; van de Ven, CP.; Okoye, B.; Smeulders, N.; Vujanic, GM.; Verschuur, A.; L'Hermine-Coulomb, A.; de Camargo, B.; de Aguirre Neto, JC.; Schenk, JP.; van den Heuvel-Eibrink, MM.; Pritchard-Jones, K.; Graf, N.; Bergeron, C.; Furtwangler, R.

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

All Authors

Sudour-Bonnange, H.

van Tinteren, H.

Ramirez-Villar, GL.

Godzinski, J.

Irtan, S.

Gessler, M.

Chowdhury, T.

Audry, G.

Fuchs, J.

Powis, M.

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LTHT Author

Powis, Mark

LTHT Department

Paediatric Surgery

Publication Date

2024

Item Type

Journal Article
Randomized Controlled Trial
Clinical Trial, Phase III

Abstract

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.