Abstract
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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Buckley KS. Pediatric genitourinary tumors. Curr Opin Oncol. 2012;24(3):291–6.
Gooskens SL, Furtwängler R, Vujanic GM, et al. Clear cell sarcoma of the kidney: a review. Eur J Cancer. 2012;48(14):2219–26.
Van Den Heuvel-Eibrink MM, van Tinteren H, Rehorst H, et al. Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group. Pediatr Blood Cancer. 2011;56(5):733–7.
Luithle T, Szavay P, Furtwängler R, et al. Treatment of cystic nephroma and cystic partially differentiated nephroblastoma—a report from the SIOP/GPOH study group. J Urol. 2007;177(1):294–6.
Davidoff AM. Wilms’ tumor. Curr Opin Pediatr. 2009;21(3):357–64.
Geller E, Kochan PS. Renal neoplasms of childhood. Radiol Clin North Am. 2011;49(4):689–709.
De Kraaker J, Graf N, Von Tinteren H, et al. Reduction of postoperative chemotherapy in children with stage I intermediate risk and anaplastic Wilms tumors (SIOP 93-01 trial): a randomised controlled trial. Lancet. 2004;364(9441):1229–35.
Reinhard H, Semler O, Büger D, et al. Results of the SIOP 93-01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms tumor. Klin Pädiatr. 2004;216(3):132–40.
Pastore G, Znaor A, Spreafico F, et al. Malignant renal tumor incidence and survival in European children. Report from the Automated Childhood Cancer Information System project. Eur J Cancer. 2006;42(13):2103–14.
Zoubek A, Kajtar P, Flucher-Wolfram B, et al. Response of untreated stage IV Wilms’ tumor to single dose carboplatin assessed by “up front” window therapy. Med Pediatr Oncol. 1995;25(1):8–11.
Urban CE, Lackner H, Schwinger W, et al. Partial nephrectomy in well-responding stage I Wilms’ tumors: report of three cases. Pediatr Hematol Oncol. 1995;12(2):143–52.
Warmann SW, Furtwängler R, Blumenstock G, et al. Tumor biology influences the prognosis of nephroblastoma patients with primary pulmonary metastases: results from SIOP 93-01/GPOH and SIOP 2001/GPOH. Ann Surg. 2011;254(1):155–62.
Fuchs J, Szavay P, Luithle T, et al. Surgical implications for liver metastases in nephroblastoma—data from the SIOP/GPOH study. Surg Oncol. 2008;17(1):33–40.
Fuchs J, Kienecker K, Furtwängler K, et al. Surgical aspects in the treatment of patients with unilateral wilms tumor: a report from the SIOP 93-01/German Society of Pediatrics Oncology and Hematology. Ann Surg. 2009;249:666–71.
Paya K, Horcher E, Rauhofer U, et al. Bilateral Wilms’ tumor—surgical aspects. Eur J Pediatr Surg. 2001;11(2):99–104.
Cozzi F, Schiavetti A, Morini F, et al. Re: partial nephrectomy for unilateral wilms tumor: results of study SIOP 93-01/GPOH. J Urol. 2004;171:2383.
Delemarre JF, Sandstedt B, Harms B, et al. The new SIOP (Stockholm) working classification of renal tumours of childhood. International Society of Paediatric Oncology. Med Pediatr Oncol. 1996;26(2):145–6.
Bernstein L, Linet M, Smith MA, et al. Renal tumors. Cancer incidence and survival among children and adolescents: 1975–1995. United States SEER Program. 1999;79–90.
Meadows AT, Friedman DL, Neglia JP, et al. Second neoplasms in survivors of childhood cancer: findings from the Childhood Cancer Survivor Study cohort. J Clin Oncol. 2009;27(14):2356–62.
Conflict of interest
The authors declare that there is no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Zimmermann, C., Pötschger, U., Amann, G. et al. 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. memo 5, 289–295 (2012). https://doi.org/10.1007/s12254-012-0057-5
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12254-012-0057-5