Kidney Tumors

The TARGET Kidney Tumor projects elucidate comprehensive molecular characterization to determine the genetic changes that drive the initiation and progression of high-risk or hard-to-treat childhood cancers. Pediatric kidney tumors fall into four primary categories: Wilms tumors (~85% of all cases), clear cell sarcomas of the kidney (~5%), congenital mesoblastic nephromas (~4%), and rhabdoid tumors of the kidney (~3%). The TARGET initiative is investigating three of these tumor types. TARGET initiated the Kidney Tumor Project to elucidate the mutation spectrums of highly aggressive forms of the disease in relapsed favorable histology Wilms tumor, anaplastic Wilms tumor, clear cell sarcoma of the kidney (CSSK) and rhabdoid tumor (RT). The tissues used in this study were collected from patients enrolled in the National Wilms Tumor Study (mostly NWTS-5) clinical trial that was completed by the Children's Oncology Group (COG).
Kidney tumors are rare and generally curable in children. However, there are subsets of patients afflicted with these diseases that do not respond to treatment or eventually relapse. These patients usually have poor clinical outcomes as compared with the majority of children diagnosed with kidney tumors. All patients undergo therapy regimens that can be detrimental later in life. Through genome-wide characterization, TARGET investigators are identifying critical molecular alterations in these tumors, mostly from relapsed patients. Future research may identify treatments tailored to these alterations to improve outcomes for children afflicted with these kidney tumors. To learn more about pediatric kidney cancers and current treatment strategies, visit the NCI childhood kidney tumor website.

The Kidney Tumor Projects have been completed; the Wilms tumor (WT), CCSK, and RT datasets are now available without restrictions on their use in publications or presentations.

Last updated: January 09, 2020