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Publications

Journal Article Ppublish

PubMed: 40545412 DOI: 10.1016/j.urolonc.2025.05.018

Outcome of patients with a first relapse after intermediate- or high-risk Wilms tumor, treated according to SIOP WT 2001/UK-IMPORT study; A report from the SIOP renal tumor study group.

Urol Oncol 43(9):525.e19-525.e32

Authors: Alissa Groenendijk, Harm van Tinteren, Ronald R de Krijger, Gordan Vujanic, Reem Al-Saadi, Kathy Pritchard-Jones, Gema L Ramírez-Villar, Patrick Melchior, Jan Godzinski, Jens-Peter Schenk, Norbert Graf, Heidi Segers, Hélène Sudour-Bonnange, Arnauld C Verschuur, Jarno Drost, Daniela Perotti, Sarah Al-Jilaihawi, Jesper Brok, Marry M van den Heuvel-Eibrink, Filippo Spreafico, Annelies M C Mavinkurve-Groothuis

Affiliations

  • Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands.
  • Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands; Department of Pathology, University Medical Center Utrecht, Utrecht, the Netherlands.
  • Department of Pathology, Sidra Medicine, Doha, Qatar; Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, Doha, Qatar.
  • Histopathology, Great Ormond Street Hospital NHS Trust for Children, London, UK; Developmental Biology & Cancer Research & Teaching Department, UCL Great Ormond Street Institute for Child Health, University College London, London UK.
  • Developmental Biology & Cancer Research & Teaching Department, UCL Great Ormond Street Institute for Child Health, University College London, London UK.
  • Department of Pediatric Oncology, Hospital Universitario Virgen del Rocío, Seville, Spain.
  • Department of Radiotherapy and Radiooncology, Saarland University Medical Center, Homburg, Germany.
  • Department of Pediatric Surgery, Marciniak Hospital, Wroclaw, Poland; Department of Pediatric Traumatology and Emergency Medicine, Medical University Wroclaw, Wroclaw, Poland.
  • Department of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Heidelberg, Germany.
  • Department of Hematology and Oncology, Saarland University Hospital, Homburg, Germany.
  • Department of Pediatric Hemato-Oncology, University Hospitals Leuven and Catholic University Leuven, Leuven, Belgium.
  • Department of Pediatric Oncology and AYA Unit, Centre Oscar Lambret, Lille, France.
  • Department of Pediatric Oncology, Hôpital d'Enfants de La Timone, Marseille, France.
  • Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands; Oncode Institute, Utrecht, the Netherlands.
  • Predictive Medicine: Molecular Bases of Genetic Risk, Department of Experimental Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
  • Department of Paediatric Oncology, Bristol Royal Hospital for Children, Bristol, UK.
  • Department of Paediatrics and Adolescent Medicine, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
  • Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands; Child Health, UMCU-Wilhelmina Children's Hospital, Utrecht, the Netherlands.
  • Pediatric Oncology Unit, Department of Medical Oncology and Hematology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

Abstract

BACKGROUND

Patients with a Wilms tumor (WT) who relapse following initial therapy with more than only vincristine and actinomycin-D are considered high-risk (group BB) or very high-risk (group CC) relapse by the International Society of Pediatric Oncology - Renal Tumor Study Group (SIOP-RTSG). We aimed to retrospectively analyze the characteristics and outcome of BB and CC patients.

METHODS

We included all patients with first relapsed WT that would currently be considered BB (n = 148) and CC (n = 72) relapse and registered in the SIOP 2001/UK-IMPORT study. We collected information on relapse treatment and calculated 5-year event-free (EFS) and overall survival (OS) rates per relapse risk group and treatment. Multivariable Cox regression analysis was performed to identify patient and tumor characteristics that were significantly associated with survival.

FINDINGS

The 5-year estimated EFS and OS rates of BB patients were 62·7% (95% CI: 54·9-71·6%) and 67·6% (95% CI: 59·8-76·4%), respectively. Five-year survival rates for the subset of BB patients treated with VAD and RT (n = 42/94) were 58·7% (95% CI: 45·0-76·6%) for EFS and 66·5% (95% CI: 53·1-83·2%) for OS. Five-year estimated EFS and rates for CC patients were 17·4% (95% CI: 10·4-29·0%) and 18·6% (95% CI: 11·3-30·5%), respectively. In multivariable Cox regression analysis, patients seemed to benefit from high dose chemotherapy and autologous stem cell rescue (HDCT) and surgery at first relapse.

INTERPRETATION

Second-line treatment was able to rescue two-thirds of BB relapse patients. In contrast, survival rates in CC patients at first relapse remain poor with conventional drugs, even with camptothecin-containing regimens. A subset of BB and CC patients may benefit from HDCT and surgery at relapse. However, to validate these findings, they must be reevaluated in future trials to eliminate bias from the analyses that is inherent to retrospective studies.

MeSH

Regression Analysis, Neoplasms, Wilms Tumor, Therapeutics, Survival Rate