Treatment of children with favorable histology Wilms tumor with extrapulmonary metastases: A report from the COG studies AREN0533 and AREN03B2 and NWTSG study NWTS-5

Benedetti DJ, Varela CR, Renfro LA, Tornwall B, Dix DB, Ehrlich PF, Glick RD, Kalapurakal J, Perlman E, Gratias E, Seibel NL, Geller JI, Khanna G, Malogolowkin M, Grundy P, Fernandez CV, Dome JS, Mullen EA. Treatment of children with favorable histology Wilms tumor with extrapulmonary metastases: A report from the COG studies AREN0533 and AREN03B2 and NWTSG study NWTS-5. Cancer. 2024 Mar 15;130(6):947-961. doi: 10.1002/cncr.35099. Epub 2023 Nov 7. PubMed PMID: 37933882; PubMed Central PMCID: PMC10922062.

Patients with Stage IV Favorable Histology Wilms Tumor (FHWT) with extrapulmonary metastases (EPM) constitute a small subset of FHWT patients. Due to their rarity and heterogeneity, optimal treatment is not well understood. COG protocol AREN0533 assigned patients with FHWT and EPM to intensified chemotherapy, Regimen M, after initial DD-4A chemotherapy. To improve understanding of prognostic factors and best therapies, we reviewed experiences of patients with EPM on AREN0533, as well as on protocols AREN03B2 and NWTS-5.