Update on Surveillance for Wilms Tumor and Hepatoblastoma in Beckwith–Wiedemann Syndrome and Other Predisposition Syndromes
Jennifer M. Kalish
Kerri D. Becktell
Gaëlle Bougeard
Garrett M. Brodeur
Lisa R. Diller
Andrea S. Doria
Jordan R. Hansford
Steven D. Klein
Wendy K. Kohlmann
Christian P. Kratz
Suzanne P. MacFarland
Kristian W. Pajtler
Surya P. Rednam
Jaclyn Schienda
Lisa J. States
Anita Villani
Rosanna Weksberg
Kristin Zelley
Gail E. Tomlinson
Jack J. Brzezinski
Abstract
Wilms tumors are commonly associated with predisposition syndromes, many but not all of which include overgrowth. Several of these syndromes also include a risk of other embryonal malignancies—particularly hepatoblastoma. Guidelines for surveillance in this population were published in 2017, and recently, members of the American Association for Cancer Research Pediatric Cancer Working Group met to update those guidelines with a review of more recently published evidence and risk estimates. This perspective serves to update pediatric oncologists, geneticists, radiologists, counselors, and other healthcare professionals on revised diagnostic criteria; review previously published surveillance guidelines; and harmonize updated surveillance recommendations in the North American and Australian contexts for patients with overgrowth syndromes and other syndromes associated with Wilms tumor predisposition.