MINISYMPOSIUM Wilms tumour: prognostic factors, staging, therapy and late effects Sue C. Kaste & Jeffrey S. Dome & Paul S. Babyn & Norbert M. Graf & Paul Grundy & Jan Godzinski & Gill A. Levitt & Helen Jenkinson Received: 22 May 2007 / Revised: 15 October 2007 / Accepted: 24 October 2007 / Published online: 17 November 2007 # Springer-Verlag 2007 Abstract Wilms tumour is the most common malignant renal tumour in children. Dramatic improvements in survival have occurred as the result of advances in anaesthetic and surgical management, irradiation and chemotherapy. Current therapies are based on trials and studies primarily conducted by large multi-institutional cooperatives including the Société Internationale d’Onco- logie Pédiatrique (SIOP) and the Children’ s Oncology Group (COG). The primary goals are to treat patients according to well-defined risk groups in order to achieve the highest cure rates, to decrease the frequency and intensity of acute and late toxicity and to minimize the cost of therapy. The SIOP trials and studies largely focus on the issue of preoperative therapy, whereas the COG trials and studies start with primary surgery. This paper reviews prognostic factors and staging systems for Wilms tumour and its current treatment with surgery and chemotherapy. Surgery remains a crucial part of treatment for nephroblas- toma, providing local primary tumour control and adequate staging and possibly controlling the metastatic spread and central vascular extension of the disease. Partial nephrec- tomy, when technically feasible, seems reasonable not only in those with bilateral disease but also in those with unilateral disease where the patient has urological disorders or syndromes predisposing to malignancy. Partial nephrec- tomy, however, is frequently not sufficient for an anaplastic variant of tumour. The late effects for Wilms tumour and its treatment are also reviewed. The treatment of Wilms tumour has been a success story, and currently in excess of 80% of children diagnosed with Wilms tumour can look forward to long-term survival, with less than 20% experi- encing serious morbidity at 20 years from diagnosis. The Pediatr Radiol (2008) 38:2–17 DOI 10.1007/s00247-007-0687-7 This work was supported in part by grants P30 CA-21765. S. C. Kaste (*) Department of Radiological Sciences, St. Jude Children’ s Research Hospital, 332 N. Lauderdale, MS #752, Memphis, TN 38105-2794, USA e-mail: sue.kaste@stjude.org J. S. Dome Department of Oncology, St. Jude Children’ s Research Hospital, Memphis, TN, USA P. S. Babyn Department of Radiology, Hospital for Sick Children, Toronto, Canada N. M. Graf Clinic for Pediatric Oncology and Hematology, University Hospital of the Saarland, Homburg, Germany P. Grundy Division of Pediatric Hematology, Oncology and Palliative Care, and Northern Alberta Children’ s Cancer Program, University of Alberta, Edmonton, Canada J. Godzinski Department of Oncological Surgery for Children and Adolescents, Mother and Child Institute, Warsaw, Poland G. A. Levitt Paediatric Oncology, Great Ormond Street Hospital for Sick Children NHS Trust, London, UK H. Jenkinson Oncology Department, Birmingham Children’ s Hospital NHS Trust, Birmingham, UK