Surveillance for Wilms tumour in at-risk individuals - pragmatic recommendations for best practice

Richard H Scott ¹, Lisa Walker ², ystein E Olsen ³, Gill Levitt ⁴, Ian Kenney ⁵, Eamonn Maher ⁶, Catherine M Owens ³, Kathryn Pritchard-Jones ⁷, Alan Craft ⁸ and Nazneen Rahman ^1*

¹ Section of Cancer Genetics, Institute of Cancer Research, Sutton, Surrey, United Kingdom
² Department of Medical Genetics, Addenbrookes Hospital, Cambridge, United Kingdom
³ Department of Radiology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom
⁴ Department of Haematology/Oncology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom
⁵ Department of Radiology, Royal Alexandra Hospital for Sick Children, Brighton, United Kingdom
⁶ Section of Medical and Molecular Genetics, Department of Paediatrics and Child Health, United Kingdom
⁷ Paediatric Oncology, Institute of Cancer Research, Sutton, Surrey, United Kingdom
⁸ Department of Paediatrics, Royal Victoria Infirmary, Newcastle-Upon-Tyne, United Kingdom

^* To whom correspondence should be addressed. E-mail: nazneen{at}icr.ac.uk.

Accepted 17 July 2006

Abstract

The majority of Wilms tumours (WT) occur in otherwise well children, but a small proportion occur in children with genetic syndromes associated with elevated risks of WT. Surveillance for WT has become widespread, despite lack of clarity about which children are at increased risk of WT, limited evidence of the efficacy of screening or guidance as to how screening should be implemented. We reviewed the available literature, which suggests that the potential risks and benefits of WT surveillance are finely balanced and that there is no clear evidence that screening reduces mortality or morbidity. Prospective, evidence-based data on the efficacy of WT screening would be difficult and costly to generate and are unlikely to become available in the foreseeable future. We have therefore produced the following pragmatic recommendations for tumour surveillance in children at risk of WT based on our review: (1) Surveillance should be offered to children at >5% risk of WT. (2) Surveillance should only be offered after review by a Clinical Geneticist. (3) Surveillance should be by renal ultrasonography every 3-4 months. (4) Surveillance should continue until 5 years in all conditions except Beckwith-Wiedemann syndrome, Simpson-Golabi-Behmel syndrome and some Familial WT pedigrees where it should continue until 7 years. (5) Surveillance can be undertaken at a local centre but should be performed by someone with experience of paediatric ultrasonography. (6) Screen-detected lesions should be managed at a specialist centre.

Keywords: Beckwith-Wiedemann syndrome, WT1, Wilms tumour, hemihypertrophy, screening

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