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Surveillance for Wilms tumour in at-risk children: pragmatic recommendations for best practice
R H Scott1, L Walker, Ø E Olsen
1Section of Cancer Genetics, Institute of Cancer Research, Sutton, Surrey, UK.
Insights
Wilms tumour surveillance is recommended for children with over a 5% risk, using renal ultrasonography every 3-4 months until age 5 or 7. Evidence for screening efficacy is limited, necessitating pragmatic guidelines for at-risk pediatric populations.
Area of Science:
- Pediatric Oncology
- Clinical Genetics
- Diagnostic Imaging
Background:
- Wilms tumour (WT) predominantly affects healthy children, but a subset arises in those with genetic predispositions.
- Current WT surveillance practices are widespread but lack clear risk stratification and evidence of efficacy.
- Uncertainty exists regarding optimal screening implementation for pediatric WT.
Purpose of the Study:
- To review the available literature on Wilms tumour surveillance.
- To formulate pragmatic recommendations for surveillance in at-risk children.
- To address the balance between risks and benefits of WT screening.
Main Methods:
- A comprehensive literature review was conducted.
- Analysis focused on identifying evidence for Wilms tumour screening efficacy.
- Recommendations were developed based on the reviewed literature.
Main Results:
- The risks and benefits of Wilms tumour surveillance are not clearly defined, with limited evidence of reduced mortality or morbidity.
- Generating prospective data on screening efficacy is challenging and unlikely in the near future.
- Current surveillance strategies require refinement due to a lack of robust evidence.
Conclusions:
- Surveillance is recommended for children with >5% WT risk, following clinical geneticist review.
- Renal ultrasonography every 3-4 months is advised until age 5 (or 7 for specific syndromes/pedigrees).
- Screening should be performed by experienced personnel, with detected lesions managed at specialist centers.
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.