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Journal of Cancer Research and Clinical Oncology

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01-12-2019 | Nephroblastoma | Original Article – Clinical Oncology

The effectiveness of Wilms tumor screening in Beckwith–Wiedemann spectrum

Authors: Alessandro Mussa, Kelly A. Duffy, Diana Carli, Jessica R. Griff, Riccardo Fagiano, Jonida Kupa, Garrett M. Brodeur, Giovanni Battista Ferrero, Jennifer M. Kalish

Published in: Journal of Cancer Research and Clinical Oncology | Issue 12/2019

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 These data were combined with unpublished data from patients in the authors’ cohorts.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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Title: The effectiveness of Wilms tumor screening in Beckwith–Wiedemann spectrum
Authors: Alessandro Mussa
Kelly A. Duffy
Diana Carli
Jessica R. Griff
Riccardo Fagiano
Jonida Kupa
Garrett M. Brodeur
Giovanni Battista Ferrero
Jennifer M. Kalish
Publication date: 01-12-2019
Publisher: Springer Berlin Heidelberg
Keywords: Nephroblastoma
Beckwith-Wiedemann Syndrome
Beckwith-Wiedemann Syndrome
Published in: Journal of Cancer Research and Clinical Oncology / Issue 12/2019
Print ISSN: 0171-5216
Electronic ISSN: 1432-1335
DOI: https://doi.org/10.1007/s00432-019-03038-3

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