The expansion of knowledge regarding driver mutations for Wilms tumor (WT) and malignant rhabdoid tumor of the kidney (MRT) and various translocations for other pediatric renal tumors opens up new possibilities for diagnosis and treatment. In addition, there are growing data surrounding prognostic factors that can be used to stratify WT treatment to improve outcomes. Here, we review the molecular landscape of WT and other pediatric renal tumors as well as WT prognostic factors. We also review incorporation of circulating tumor DNA/liquid biopsies to leverage this molecular landscape, with potential use in the future for distinguishing renal tumors at the time of diagnosis and elucidating intratumor heterogeneity, which is not well evaluated with standard biopsies. Incorporation of liquid biopsies will require longitudinal collection of multiple biospecimens. Further preclinical research, identification and validation of biomarkers, molecular studies, and data sharing among investigators are crucial to inform therapeutic strategies that improve patient outcomes.
Pediatric blood & cancer. 2023 Jan 02 [Epub ahead of print]
Amy L Walz, Mariana Maschietto, Brian Crompton, Nicholas Evageliou, David Dix, Godelieve Tytgat, Manfred Gessler, David Gisselsson, Najat C Daw, Jenny Wegert
Division of Hematology, Oncology, Neuro-Oncology, and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA., Research Center, Boldrini Children's Hospital, Campinas, São Paulo, Brazil., Department of Pediatric Oncology, Dana-Farber/Harvard Cancer Center, Dana Farber Cancer Institute, Boston, Massachusetts, USA., Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., British Columbia Children's Hospital, Vancouver, British Columbia, Canada., Princess Máxima Center for Pediatric Oncology, CS Utrecht, The Netherlands., Comprehensive Cancer Center Mainfranken, Wuerzburg, Germany., Cancer Cell Evolution Unit, Division of Clinical Genetics, Department of Laboratory Medicine, Lund University, Lund, Sweden., Division of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA., Theodor-Boveri-Institute/Biocenter, Developmental Biochemistry, University of Wuerzburg, Wuerzburg, Germany.