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

Autor: Sudour-Bonnange H; Department of Children and AYA Oncology, Centre Oscar Lambret, Lille, France. h-sudour@o-lambret.fr., van Tinteren H; Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands., Ramírez-Villar GL; Department of Pediatric Oncology, Hospital Universitario Virgen Del Rocio, Sevilla, Spain., Godzinski J; Department of Pediatric Surgery, Marciniak Hospital, Fieldorfa 2 and Department of Pediatric Traumatology and Emergency Medicine, Wroclaw Medical university, Wrocław, Poland., Irtan S; Department of Pediatric Surgery, Hospital Trousseau, Paris, France., Gessler M; Theosor-Boveri Institute/Biocenter and Comprehensive Cancer Center Mainfranken, University of Wuerzburg, Würzburg, Germany., Chowdhury T; Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK., Audry G; Department of Pediatric Surgery, Hospital Trousseau, Paris, France., Fuchs J; Department of Pediatric Surgery and Urology, University Hospital Tübingen, 72076, Tuebingen, Germany., Powis M; Department of Pediatric Surgery, Leeds Teaching Hospital NHS Trust, Leeds, UK., van de Ven CP; Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands., Okoye B; Department of Paediatric Surgery, St George's University Hospital, London, UK., Smeulders N; Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK., Vujanic GM; Department of Pathology, Sidra Medicine and weill Cornell Medicine- Qatar, Doha, Qatar., Verschuur A; Department of Pediatric Hemato-Oncology, Hospital La Timone, Marseille, France., L'Herminé-Coulomb A; Department of Pathology; Hospital Trousseau, Paris, France., de Camargo B; Pediatric Hematology and Oncology program, research Center, Instituto National de Cancer, Rio de Janeiro, Brazil., de Aguirre Neto JC; Department of Pediatric Hematology and Oncology, Hospital Santa Casa Belo-Horizonte, Belo Horizonte, Brazil., Schenk JP; Sektion Pädiatrische Radiologie, Klinik für Diagnostische und Interventionelle Radiologie, Universitätsklinikum Heidelberg, Heidelberg, Deutschland., van den Heuvel-Eibrink MM; Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands.; Division of Childhealth, Wilhelmina Children's Hospital, Utrecht, the Netherlands., Pritchard-Jones K; Developmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK., Graf N; Saarland University, Homburg, Germany., Bergeron C; Department of Pediatric Oncology and Hematology, Centre Léon Berard, Lyon, France., Furtwängler R; Saarland University, Homburg, Germany.; Division of Pediatric Hematology and Oncology, Department of Pediatrics, Inselspital, University Bern, Bern, Switzerland.
Jazyk: angličtina
Zdroj: British journal of cancer [Br J Cancer] 2024 Oct; Vol. 131 (6), pp. 972-981. Date of Electronic Publication: 2024 Jul 30.
DOI: 10.1038/s41416-024-02799-0
Abstrakt: 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.
(© 2024. The Author(s), under exclusive licence to Springer Nature Limited.)
Databáze: MEDLINE
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