Treatment of stage I anaplastic Wilms’ tumour: a report from the Children’s Oncology Group AREN0321 study

Daw NC, Chi YY, Kim Y, Mullen EA, Kalapurakal JA, Tian J, Khanna G, Geller JI, Perlman EJ, Ehrlich PF, Warwick AB, Grundy PE, Fernandez CV, Dome JS. Treatment of stage I anaplastic Wilms’ tumour: a report from the Children’s Oncology Group AREN0321 study. Eur J Cancer. 2019 Sep;118:58-66. doi: 10.1016/j.ejca.2019.05.033. Epub 2019 Jul 17. PubMed PMID: 31325873; PubMed Central PMCID: PMC6690766.

In the fifth National Wilms Tumor Study (NWTS-5), the 4-year event-free (EFS) and overall survival (OS) estimates for 29 patients with stage I focal (n=10) or diffuse (n= 19) anaplastic Wilms tumor (AWT) treated with vincristine and dactinomycin without flank radiation were 69.5% and 82.6%, respectively. The Children’s Oncology Group AREN0321 study evaluated whether adding doxorubicin and flank radiation improves survival for these patients. Tumor histology and stage were confirmed by real-time central pathology, surgery, and radiology review. Patients received 25 weeks of vincristine, dactinomycin and doxorubicin (cumulative dose 150 mg/m2) with flank radiation (1080 cGy). We retrospectively analyzed outcomes of all patients with stage I AWT enrolled on National Wilms Tumor Studies (NWTS) 1–5 and AREN0321 with respect to treatment regimens.