Ehrlich PF, Chi YY, Chintagumpala MM, Hoffer FA, Perlman EJ, Kalapurakal JA, Tornwall B, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Grundy PE, Fernandez CV, Dome JS. Results of Treatment for Patients With Multicentric or Bilaterally Predisposed Unilateral Wilms Tumor (AREN0534): A report from the Children’s Oncology Group. Cancer. 2020 Aug 1;126(15):3516-3525. doi: 10.1002/cncr.32958. Epub 2020 May 27. PubMed PMID: 32459384; PubMed Central PMCID: PMC7769115.
Study ID Citation
Abstract
A primary aim of Children’s Oncology Group (COG) study AREN0534 was to facilitate partial nephrectomy in 25% of children with bilaterally-predisposed unilateral tumors (WAGR, multifocal and overgrowth syndromes). The purpose of this prospective study was to achieve an excellent EFS and OS, while preserving renal tissue through pre-operative chemotherapy, completing definitive surgery by 12 weeks from diagnosis, and modifying post-operative chemotherapy based on histologic response. The treating institution identified if a predisposition syndrome existed. Patients had central review of imaging studies through the biology and classification study AREN03B2 and then were eligible to enroll on AREN0534. Patients were treated with induction chemotherapy determined by localized or metastatic disease on imaging (and histology if a biopsy had been undertaken). Surgery was based on radiographic response at 6 or 12 weeks. Further chemotherapy was determined by histology. Favorable Histology Stage III or IV disease patients received radiotherapy as well as Stage I-IV with anaplasia.