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Risk of Wilms' tumour with multicystic kidney disease: a systematic review
1Paediatric Department, Sandwell General Hospital, West Bromwich B71 4HJ, UK. hassibnarchi@hotmail.com
Insights
Children with multicystic kidney disease (MCKD) have a very low risk of developing Wilms' tumour. Current follow-up strategies for MCKD are not evidence-based and may not be necessary.
Area of Science:
- Pediatric Nephrology
- Oncology
- Medical Genetics
Background:
- Children with multicystic kidney disease (MCKD) are often managed conservatively with prolonged follow-up due to perceived Wilms' tumour risk.
- This perceived risk is poorly defined and lacks robust evidence, leading to uncertainty in management strategies.
Purpose of the Study:
- To systematically review the literature and estimate the risk of Wilms' tumour development in children with unilateral MCKD managed conservatively.
- To evaluate the evidence supporting current follow-up protocols for children with MCKD.
Main Methods:
- Systematic review of published prospective and retrospective cohort studies.
- Inclusion criteria: children diagnosed with unilateral MCKD and managed conservatively.
- Exclusion criteria: bilateral MCKD, nephrectomy for reasons other than malignancy. Wilms' tumour incidence was estimated using Poisson distribution.
Main Results:
- Analysis of 26 studies involving 1041 eligible children with unilateral MCKD.
- No cases of Wilms' tumour were reported in the studied population.
- The estimated upper 97.5% confidence interval for Wilms' tumour development was 0.0035 (3.5 per 1000 children).
Conclusions:
- The evidence does not support the necessity of current ultrasound follow-up modalities for children with MCKD.
- Establishing national or European registries could improve the precision of Wilms' tumour risk estimates for MCKD patients.
- Further research is needed to define optimal management and follow-up for children with MCKD.
Background:
Children with multicystic kidney disease (MCKD) are increasingly managed conservatively and are followed up throughout childhood because they are perceived to be at increased risk of developing Wilms' tumour. With this risk still poorly defined and somewhat controversial, the strategy and the duration of follow up do not seem to be based on evidence.
Methods:
Systematic review of the literature for all published cohort studies (prospective and retrospective) of children diagnosed to have unilateral MCKD and managed conservatively.
Exclusion Criteria:
bilateral MCKD, nephrectomy (not for malignancy) during the follow up period. We estimated for children with MCKD the probability of developing Wilms' tumour during the follow up period, with 95% CI using the Poisson distribution.
Results:
From 26 reviewed studies, no cases of Wilms' tumour developed in 1041 eligible children. The mean probability of a child with unilateral MCKD to develop Wilms' was therefore nil, with a 97.5% upper CI estimated at 0.0035 (or 3.5 per 1000 children).
Conclusion:
The development of a national or a European registry for children with MCKD would increase the precision of their risk estimate to develop Wilms' tumour. In the meantime, there is no evidence to support any of the different modalities for following up these children by ultrasound, if indeed such a strategy is necessary.
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