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WT1-mutant Wilms tumors exhibit a high rate of concomitant CTNNB1 mutations, associated with activated Wnt signaling. Here, we show by laser and manual microdissection of different histologic cell types from 6 WT1-mutant tumor samples that 1 patient's tumor can contain up to 4 distinct mutations in CTNNB1 and/or WTX. Consecutive sections may also harbor different CTNNB1 mutations. The variability of activating CTNNB1 mutations demonstrates the multifocal nature of WT1-mutant Wilms tumors. As multiple independent tumors can occur in patients with constitutional WT1 mutations, they need to be surveyed more closely for tumor development. Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.


Constanze Duhme, Maike Busch, Eva Heine, Carmen de Torres, Jaume Mora, Brigitte Royer-Pokora. WT1-Mutant Wilms Tumor Progression Is Associated With Diverting Clonal Mutations of CTNNB1. Journal of pediatric hematology/oncology. 2021 Mar 01;43(2):e180-e183

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PMID: 31876779

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