Screening for Wilms tumor in children with Beckwith-Wiedemann syndrome or idiopathic hemihypertrophy
✍ Scribed by Choyke, Peter L.; Siegel, Marilyn J.; Craft, Alan W.; Green, Daniel M.; DeBaun, Michael R.
- Publisher
- John Wiley and Sons
- Year
- 1999
- Tongue
- English
- Weight
- 78 KB
- Volume
- 32
- Category
- Article
- ISSN
- 0098-1532
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✦ Synopsis
Background:
Children with beckwith-wiedemann syndrome and idiopathic hemihypertrophy (bws/hh) are at increased risk for developing wilms tumor and screening with abdominal sonography is frequently recommended. however, there is a paucity of published data supporting this strategy. the purpose of this study was to determine whether sonographic screening at intervals of 4 months or less reduced the proportion of late-stage wilms tumor (wt) in children with bws/hh.
Procedure:
A case series analysis was employed to compare the proportion of late-stage (stage iii or iv) wilms tumor in patients with bws/hh who were screened with sonography (n = 15) to the proportion of late-stage wilms tumor in unscreened patients with bws/hh (n = 59). patients were identified from the bws registry and from previously published studies. screened patients had sonograms at intervals of 4 months or less.
Results:
None of the 12 screened children with wilms tumor had late-stage disease, whereas 25 of 59 (42%) of unscreened children had late-stage wilms tumor, a difference that was statistically significant (p < 0.003). three children had false positive screening studies. they were operated on for suspected wilms tumor but the lesions proved to be complicated renal cysts (n = 2) or nephroblastomatosis (n = 1).
Conclusions:
This study suggests that children with bws/hh may benefit from screening sonograms at intervals of 4 months or less. however, false positive screening exams may result in unnecessary surgery. given the rarity of bws/hh, a larger, prospective international screening study is necessary to determine if the benefits of screening outweigh the risks.