Forty-Five patient-derived xenografts capture the clinical and biological heterogeneity of Wilms tumor

The lack of model systems has limited the preclinical testing of novel therapies for Wilms tumor (WT) patients who have poor outcomes. Herein, we established 45 heterotopic WT patient-derived xenografts (WTPDX) in CB17 scid-/- mice that capture the biological heterogeneity of Wilms tumor (WT). These WTPDX include 6 from patients with diffuse anaplasia, 9 from patients who later experienced disease relapse, and 13 from patients with bilateral disease. Early passage WTPDX showed enrichment of blastemal gene expression. Favorable histology WTPDX were chemosensitive, whereas unfavorable histology WTPDX were resistant to conventional chemotherapy with vincristine, actinomycin-D, and doxorubicin given singly or in combination. This WTPDX library is a unique scientific resource that retains the spectrum of biological heterogeneity present in WT and provides an essential tool to test novel targeted therapies in the era of precision medicine.

Type: Other
Archiver: European Genome-Phenome Archive (EGA)

2 Datasets 3 Publications

Click on a Dataset ID in the table below to learn more, and to find out who to contact about access to these data

Dataset ID	Description	Technology	Samples
EGAD00001004506	WES files for CHEN WTPDX paper titled "Forty-Five patient-derived xenografts capture the clinical and biological heterogeneity of Wilms tumor"	Illumina HiSeq 2000	107
EGAD00001004507	RNAseq files for CHEN WTPDX RNASEQ paper titled "Forty-Five patient-derived xenografts capture the clinical and biological heterogeneity of Wilms tumor"	Illumina HiSeq 2000	88

Publications	Citations
Forty-five patient-derived xenografts capture the clinical and biological heterogeneity of Wilms tumor. Murphy AJ, Chen X, Pinto EM, Williams JS, Clay MR, Pounds SB, Cao X, Shi L, Lin T, Neale G, Morton CL, Woolard MA, Mulder HL, Gil HJ, Rehg JE, Billups CA, Harlow ML, Dome JS, Houghton PJ, Easton J, Zhang J, George RE, Zambetti GP, Davidoff AM. Nat Commun 10: 2019 5806	22
<i>TERT</i> Expression in Wilms Tumor Is Regulated by Promoter Mutation or Hypermethylation, WT1, and N-MYC. Jablonowski CM, Gil HJ, Pinto EM, Pichavaram P, Fleming AM, Clay MR, Hu D, Morton CL, Pruett-Miller SM, Hansen BS, Chen X, Jones KMD, Liu Y, Ma X, Yang J, Davidoff AM, Zambetti GP, Murphy AJ. Cancers (Basel) 14: 2022 1655	3
Modeling high-risk Wilms tumors enables the discovery of therapeutic vulnerability. Ma G, Gao A, Chen J, Liu P, Sarda R, Gulliver J, Wang Y, Joiner C, Hu M, Kim EJ, Yeger H, Le HD, Chen X, Li WJ, Xu W. Cell Rep Med 5: 2024 101770	0