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TARGET - TARGET Kidney Projects Publications

TARGET Kidney Projects Publications

Program Description

The TARGET kidney projects include three different high risk tumors of the kidney. 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). TARGET investigators analyzed tumors from patients with anaplasia at diagnosis, or those who relapsed and for whom standard therapies were ineffective in order to identify biomarkers that correlate with poor clinical outcome and/or new therapeutic approaches.

Wilms tumor (WT) (Walz et al. 2015, Perlman et al. 2015, Gadd et al. 2017) is the most common type of childhood kidney cancer and usually occurs before the age of six years. Most cases present with a favorable histology and respond well to standard treatment, however there are patients who relapse unexpectedly.

A subset of WT patients are diagnosed with anaplasia (Ooms et al. 2016), an unfavorable histology WT which does not respond to current therapies.

Malignant Rhabdoid Tumors (MRT) (Chun et al. 2016, Chun et al. 2019) are rare, highly lethal tumors that most commonly present in the kidney of infants. They may also present within the brain, and these are classified as atypical teratoid/rhabdoid tumors. Very rarely, MRT may develop in the soft tissue.

Clear Cell Sarcoma of the Kidney (CCSK) (Gooskens et al. 2015) is a very rare sarcoma seldom seen outside the kidney. It has an annual incidence of approximately 20 cases in the United States. Patients present at a mean age of 3 years, and males are noticed to be affected more frequently than females. While previously considered to be of "unfavorable" histology, when patients are given the currently recommended therapies, the prognosis has greatly improved, particularly for those that are low stage. Patients with stage 3 and 4 disease continue to have a poor outcome.