Jenny Wegert, Anne Kristin Fischer, Balazs Palhazi, Taryn D Treger, Cäcilia Hilgers, Barbara Ziegler, Hyunchul Jung, Eva Jüttner, Andreas Waha, Jörg Fuchs, Steven W Warmann, Michael C Frühwald, Jochen Hubertus, Kathy Pritchard-Jones, Norbert Graf, Sam Behjati, Rhoikos Furtwängler, Manfred Gessler, Christian Vokuhl
Wilms tumors (WTs) are histologically diverse childhood cancers with variable contributions of blastema, stroma, and epithelia. A variety of cancer genes operate in WTs, including the tripartite-motif-containing-28 gene (TRIM28). Case reports and small case series suggest that TRIM28 mutations are associated with epithelial morphology and WT predisposition. Here, we systematically investigated the prevalence of TRIM28 inactivation and predisposing mutations in a cohort of 126 WTs with >2/3 epithelial cells, spanning 20 years of biobanking in the German SIOP93-01/GPOH and SIOP2001/GPOH studies...
October 4, 2023: Journal of Pathology