Journal Article (Review Article)

DKFZ-2024-02192

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Update on surveillance guidelines in emerging Wilms tumor predisposition syndromes.

Brzezinski, J. J. ; Becktell, K. D. ; Bougeard, G. ; Brodeur, G. M. ; Diller, L. R. ; Doria, A. S. ; Hansford, J. R. ; Kohlmann, W. K. ; Kratz, C. P. ; MacFarland, S. P. ; Pajtler, K.DKFZ* ; Rednam, S. P. ; Schienda, J. ; States, L. J. ; Villani, A. ; Weksberg, R. ; Zelley, K. ; Tomlinson, G. E. ; Kalish, J. M.

2025
AACR Philadelphia, Pa. [u.a.]

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Please use a persistent id in citations: doi:10.1158/1078-0432.CCR-24-2488

Abstract: Wilms tumors are commonly associated with predisposition syndromes. Many of these syndromes are associated with specific phenotypic features and are discussed in the related paper from the AACR Pediatric Cancer Working Group. Guidelines for surveillance in this population were published in 2017 but since then several studies have identified new genes with recurrent pathogenic variants associated with increased risk for Wilms tumor development. In general, variants in these genes are less likely to be associated with other phenotypic features. Recently, members of the AACR Pediatric Cancer Working Group met to update surveillance guidelines for patients with a predisposition to Wilms tumors with a review of recently published evidence and risk estimates. Risk estimates for Wilms tumor for the more recently described genes are discussed here along with suggested surveillance guidelines for these populations. Several other emerging clinical scenarios associated with Wilms tumor predisposition are also discussed including patients with family histories of Wilms tumor and no identified causative gene, patients with bilateral tumors, and patients with somatic mosaicism for chromosome 11p15.5 alterations. This perspective serves to update pediatric oncologists, geneticists, radiologists, counselors and other healthcare professionals on emerging evidence and harmonize updated surveillance recommendations in the North American and Australian context for patients with emerging forms of Wilms tumor predisposition.

Note: 2025 Jan 6;31(1):18-24

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Contributing Institute(s):

1. B062 Pädiatrische Neuroonkologie (B062)
2. DKTK HD zentral (HD01)

Research Program(s):

1. 312 - Funktionelle und strukturelle Genomforschung (POF4-312) (POF4-312)

Appears in the scientific report 2024

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Database coverage:
; BIOSIS Previews ; Biological Abstracts ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Essential Science Indicators ; IF >= 10 ; JCR ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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