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Wilms tumour: prognostic factors, staging, therapy and late effects

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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’Oncologie 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 nephroblastoma, providing local primary tumour control and adequate staging and possibly controlling the metastatic spread and central vascular extension of the disease. Partial nephrectomy, 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 nephrectomy, 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% experiencing serious morbidity at 20 years from diagnosis. The late complications are a consequence of the type and intensity of treatment required, which in turn reflects the nature and extent of the original tumour. Continual international trial development and participation will improve matching of treatment needs with prognosis, reducing long-term complications in the majority. The advent of molecular markers of disease severity and improved functional imaging might help.

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Authors and Affiliations

  1. Department of Radiological Sciences, St. Jude Children’s Research Hospital, 332 N. Lauderdale, MS #752, Memphis, TN, 38105-2794, USA

    Sue C. Kaste

  2. Department of Oncology, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Jeffrey S. Dome

  3. Department of Radiology, Hospital for Sick Children, Toronto, Canada

    Paul S. Babyn

  4. Clinic for Pediatric Oncology and Hematology, University Hospital of the Saarland, Homburg, Germany

    Norbert M. Graf

  5. Division of Pediatric Hematology, Oncology and Palliative Care, and Northern Alberta Children’s Cancer Program, University of Alberta, Edmonton, Canada

    Paul Grundy

  6. Department of Oncological Surgery for Children and Adolescents, Mother and Child Institute, Warsaw, Poland

    Jan Godzinski

  7. Paediatric Oncology, Great Ormond Street Hospital for Sick Children NHS Trust, London, UK

    Gill A. Levitt

  8. Oncology Department, Birmingham Children’s Hospital NHS Trust, Birmingham, UK

    Helen Jenkinson

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  1. Sue C. Kaste
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Correspondence to Sue C. Kaste.

Additional information

This work was supported in part by grants P30 CA-21765.

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Kaste, S.C., Dome, J.S., Babyn, P.S. et al. Wilms tumour: prognostic factors, staging, therapy and late effects. Pediatr Radiol 38, 2–17 (2008). https://doi.org/10.1007/s00247-007-0687-7

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  • DOI: https://doi.org/10.1007/s00247-007-0687-7

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