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Surveillance for Wilms tumour in at-risk children: pragmatic recommendations for best practice
  1. R H Scott1,
  2. L Walker2,
  3. Ø E Olsen3,
  4. G Levitt4,
  5. I Kenney5,
  6. E Maher6,
  7. C M Owens3,
  8. K Pritchard-Jones7,
  9. A Craft8,
  10. N Rahman1
  1. 1Section of Cancer Genetics, Institute of Cancer Research, Sutton, Surrey, UK
  2. 2Department of Medical Genetics, Addenbrookes Hospital, Cambridge, UK
  3. 3Department of Radiology, Great Ormond Street Hospital for Children NHS Trust, London, UK
  4. 4Department of Haematology and Oncology, Great Ormond Street Hospital for Children NHS Trust
  5. 5Department of Radiology, Royal Alexandra Hospital for Sick Children, Brighton, UK
  6. 6Section of Medical and Molecular Genetics, Department of Paediatrics and Child Health, University of Birmingham, Birmingham, UK
  7. 7Paediatric Oncology, Institute of Cancer Research
  8. 8Department of Paediatrics, Royal Victoria Infirmary, Newcastle Upon Tyne, UK
  1. Correspondence to:
    N Rahman
    Section of Cancer Genetics, Brookes Lawley Building, Institute of Cancer Research, 15 Cotswold Road, Sutton, Surrey SM2 5NG, UK;nazneen.rahman{at}icr.ac.uk

Abstract

Background: Most Wilms tumours occur in otherwise healthy children, but a small proportion occur in children with genetic syndromes associated with increased risks of Wilms tumour. Surveillance for Wilms tumour has become widespread, despite a lack of clarity about which children are at increased risk of these tumours and limited evidence of the efficacy of screening or guidance as to how screening should be implemented.

Methods: The available literature was reviewed.

Results: The potential risks and benefits of Wilms tumour surveillance are finely balanced and there is no clear evidence that screening reduces mortality or morbidity. Prospective evidence-based data on the efficacy of Wilms tumour screening would be difficult and costly to generate and are unlikely to become available in the foreseeable future.

Conclusions: The following pragmatic recommendations have been formulated for Wilms tumour surveillance in children at risk, based on our review: (1) Surveillance should be offered to children at >5% risk of Wilms tumour. (2) Surveillance should only be offered after review by a clinical geneticist. (3) Surveillance should be carried out by renal ultrasonography every 3–4 months. (4) Surveillance should continue until 5 years of age in all conditions except Beckwith–Wiedemann syndrome, Simpson–Golabi–Behmel syndrome and some familial Wilms tumour pedigrees where it should continue until 7 years. (5) Surveillance can be undertaken at a local centre, but should be carried out by someone with experience in paediatric ultrasonography. (6) Screen-detected lesions should be managed at a specialist centre.

  • WAGR, Wilms tumour-aniridia-genitourinary-mental retardation

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Footnotes

  • Published Online First 20 July 2006

  • This research was supported by Institute of Cancer Research (UK). RHS is supported by the Kadoorie Charitable Foundation.

  • Competing interests: None.

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