Results of children with renal tumors treated in the Austrian–Hungarian Wilms Tumor Study 1989 and the International Society of Pediatric Oncology (SIOP) 93-01/GPOH trial in Austria

The overall outcome for children with nephroblastomas is excellent when treated according to protocols that include surgery, chemotherapy, and, in selected cases, radiotherapy. This study was conducted to provide population-based data on Austrian children who had been treated in neoadjuvant nephroblastoma trials between October 1988 and July 2001. One hundred and forty-eight children with newly diagnosed untreated renal tumors were registered in the Austrian–Hungarian Wilms Tumor Study (AHWTS) 1989 ( N = 63) and the SIOP 93-01/GPOH Study ( N = 85). After a median follow-up of 11.8 years from diagnosis, 130 patients have been reported to be alive; 18 patients have died. Three patients had carcinoma and three had nephroblastomatosis; the 5-year event-free survival (EFS) and overall survival (OS) rates for the remaining 142 patients were 83 ± 3 % and 91 ± 2 %, respectively (SIOP 93-01/GPOH trial, 5-year EFS 85 ± 4 %, OS 95 ± 2 %; AHWTS 1989 trial, 5-year EFS 79 ± 5 %, OS 86 ± 5 %). Outcome was better for low-risk pathology ( N = 14; 5-year EFS 92 ± 8 %, OS 100 %) than for intermediate-risk ( N = 106; 5-year EFS 89 ± 3 %, OS 94 ± 2 %) and high-risk pathology ( N = 22; 5-year EFS 50 ± 11 %, OS 68 ± 10 %; P < 0.001). The stage of the disease was correlated with outcome; stages I–III ( N = 102; 5-year EFS 90 ± 3 %, OS 95 ± 2 %), stage IV ( N = 25; 5-year EFS 56 ± 10 %, OS 76 ± 9 %), and stage V ( N = 15; 5-year EFS 80 ± 10 %, OS 87 ± 9 %; P = 0.008). On the basis of the expected incidence for renal tumors in childhood, these data provide evidence that almost all children with these diseases in Austria had been recruited in the respective treatment trials (mean: 12 patients/year); and the outcomes are comparable to international results.