Abstract
Purpose
It is well documented that patients with Beckwith–Wiedemann spectrum (BWS) have a significantly higher risk of developing Wilms tumor (WT) than the general population. There has been little research on the timing of WT diagnosis in BWS in regard to optimizing suggested screening protocols.
Methods
A literature search was performed to identify all reports of patients with BWS and WT. These data were combined with unpublished data from patients in the authors’ cohorts. Age at WT diagnosis was compared against data collected through the NIH Surveillance, Epidemiology, and End Results Program (SEER) registry.
Results
Patients with BWS had a significantly higher incidence of WT diagnoses between age 12 and 84 months compared to patients in the SEER registry. Patients with BWS and WT diagnosed through screening had significantly lower stages at diagnosis compared to patients with BWS that were not screened.
Conclusions
Screening until age 7 years is effective in detecting close to 95% of all WT in patients with BWS.
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Acknowledgements
We would like to thank the patients and their families for their active participation in this research. We would also like to thank our colleagues for their help in caring for these patients. We also thank Joshua Schiffman, Logan Spector, and Michaela Richardson for their help interfacing with the SEER database. This research was funded by National Institute of Health, Grant/Award Number: K08CA193915; Alex’s Lemonade Stand Foundation; and St. Baldrick’s Foundation Scholar Award, MIUR PRIN (Ministero dell’Istruzione, dell’Università e della Ricerca, Progetti di Ricerca di Interesse Nazionale) 2015 prot. 2015JHLY35 (to GBF).
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Mussa, A., Duffy, K.A., Carli, D. et al. The effectiveness of Wilms tumor screening in Beckwith–Wiedemann spectrum. J Cancer Res Clin Oncol 145, 3115–3123 (2019). https://doi.org/10.1007/s00432-019-03038-3
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DOI: https://doi.org/10.1007/s00432-019-03038-3