Gene expression data comparing Wilms tumor primary samples to patient-derived xenografts

The lack of model systems limits the preclinical testing of novel therapies to address Wilms tumor patient groups with poor outcomes. Therefore, we established 45 heterotopic Wilms tumor patient-derived xenografts (WTPDX) in CB17 scid-/- mice that capture the biological heterogeneity of Wilms tumor (WT). These WTPDX include six showing diffuse anaplasia, nine from patients who went on to experience disease relapse, and thirteen from patients with bilateral disease. WTPDX retained the genetic alterations and the global transcriptomic and methylation profile of corresponding primary WT. In addition, favorable histology WTPDX were chemosensitive, while unfavorable histology WTPDX were resistant to conventional chemotherapy with vincristine, actinomycin-D, and doxorubicin. This WTPDX library is a unique scientific resource that retains the spectrum of biological heterogeneity present in WT and provides an essential tool for the testing of novel targeted therapies in the era of precision medicine. Total RNA was extracted from 16 selected Wilms tumor patient-derived xenografts, 13 available corresponding primary tumor specimens, and three normal kidney specimens using the Qiagen RNeasy Midi kit (Qiagen, Hilden, Germany). Commercially available pooled total RNA from four human fetal kidneys was also included (Takara, Kusatsu, Japan).