Table 1.
Key Findings of Recent SIOP and NWTS/COG Renal Tumor Studies
| Study | Key Findings |
|---|---|
| NWTS-5 (1995-2002) | With surgery only, 5-year RFS rate for stage I FH WT, age < 2 years, and tumor < 550 g was 84%, leading to early study closure; 5-year OS was 98%10,11 Combined LOH at 1p and 16q predicted decreased RFS and OS for stage I-IV FH WT (2005)6 RFS and OS inferior for stage I anaplastic WT compared with stage I FH WT7 Best reported EFS and OS to date using VCR/DOX/CYCLO/ETOP for stage II-IV diffuse anaplastic WT7 |
| COG AREN0321 (2006-2013) | VCR/irinotecan produced high response rate in a phase II study of newly diagnosed stage IV anaplastic WT12 Patients with stage II-IV diffuse anaplastic WT treated with VCR/DOX/CYCLO/CARBO/ETOP (plus irinotecan for stage IV with phase II response) had EFS superior to NWTS-5, but with greater toxicity13 |
| COG AREN0532 (2006-2013) | Re-evaluation of surgery only for stage I FH, age < 2 years, tumor < 550 g; results to be reported in 2015 |
| COG AREN0533 (2007-2013) | Patients with stage IV FH WT with incomplete lung nodule response after 6 weeks of VCR/AMD/DOX showed superior EFS with the addition of CYCLO/ETOP compared with the estimated historical standard; results for patients with complete response treated without XRT will be reported in 2015 Results for patients with stage I-IV FH WT with combined LOH at 1p and 16q treated with additional chemotherapy will be reported in 2015 |
| SIOP 93-01 (1993-1999) | Patients with stage I intermediate-risk WT did just as well with 4 weeks of postoperative VCR/AMD as with 18 weeks14 Postchemotherapy histology predicted relapse, with inferior outcomes for patients with blastemal-type tumors15 Lung XRT can be omitted from treatment of patients with stage IV WT if they have a complete response to chemotherapy and/or metastasectomy16 |
| SIOP-2001 (2001-2012) | Stage I-III blastemal-type WT had superior EFS (and OS for stage I) compared with historical controls when treated with intensified chemotherapy17 Stage II-III intermediate-risk WT did not have significant differences in EFS or OS when treated without doxorubicin18 Stage IV WT with CT-only lung nodules had intermediate survival rates between those of children with normal CT scan of thorax and those with chest x-ray–detectable metastatic disease19 |
Abbreviations: AMD, dactinomycin; CARBO, carboplatin; COG, Children's Oncology Group; CT, computed tomography; CYCLO, cyclophosphamide; DOX, doxorubicin; EFS, event-free survival; ETOP, etoposide; FH, favorable histology; LOH, loss of heterozygosity; NWTS, National Wilms Tumor Study; OS, overall survival; RFS, recurrence-free survival; SIOP, International Society of Pediatric Oncology; WT, Wilms tumor; VCR, vincristine; XRT, radiation therapy.