Impact of the First Generation of Children’s Oncology Group Clinical Trials on Clinical Practice for Wilms Tumor

Dome JS, Mullen EA, Dix DB, Gratias EJ, Ehrlich PF, Daw NC, Geller JI, Chintagumpala M, Khanna G, Kalapurakal JA, Renfro LA, Perlman EJ, Grundy PE, Fernandez CV. Impact of the First Generation of Children’s Oncology Group Clinical Trials on Clinical Practice for Wilms Tumor. J Natl Compr Canc Netw. 2021 Aug 1;19(8):978-985. doi: 10.6004/jnccn.2021.7070. Review. PubMed PMID: 34416705; PubMed Central PMCID: PMC8805512.

Refinements in surgery, radiation therapy, and chemotherapy since the mid-20th century have resulted in a survival rate exceeding 90% for patients with Wilms tumor (WT). Although this figure is remarkable, a significant proportion of patients continue to have event-free survival (EFS) estimates of <75%, and nearly 25% of survivors experience severe chronic medical conditions. The first-generation Children’s Oncology Group (COG) renal tumor trials (AREN ‘0’), which opened to enrollment in 2006, focused on augmenting treatment regimens for WT subgroups with predicted EFS <75% to 80%, including those with the adverse prognostic marker of combined loss of heterozygosity (LOH) at chromosomes 1p/16q, pulmonary metastasis with incomplete lung nodule response after 6 weeks of chemotherapy, bilateral disease, and anaplastic histology. Conversely, therapy was reduced for patient subgroups with good outcomes and potential for long-term toxicity, such as those with lung metastasis with complete lung nodule response after 6 weeks of chemotherapy. This article summarizes the key findings of the first-generation COG renal tumor studies and their implications for clinical practice.