Nephroblastoma – 30-Years Period of its Treatment in the University Hospital Motol, Prague

Česká verzia

Authors: J. Mališ ¹; K. Švojgr ¹; K. Pýcha ²; V. Jeřábková ¹; S. Cyprová ¹; M. Churáčková ¹; V. Šmelhaus ¹; J. Radvanský ³; M. Zítková ⁴; R. Kodet ⁵; D. Kodetová ⁵; B. Malinová ⁶; J. Koutecký ¹; J. Šnajdauf ²; J. Starý ¹
Authors place of work: Klinika dětské hematologie a onkologie 2. LF UK a FN v Motole, Praha ¹; Klinika dětské chirurgie 2. LF UK a FN v Motole, Praha ²; Oddělení tělovýchovného lékařství, FN v Motole, Praha ³; Klinika zobrazovacích metod 2. LF UK a FN v Motole, Praha ⁴; Ústav patologie a molekulární medicíny 2. LF UK a FN v Motole, Praha ⁵; Radioterapeuticko‑onkologické oddělení, FN v Motole, Praha ⁶
Published in the journal: Klin Onkol 2013; 26(5): 336-342
Category: Original Articles

Summary

Introduction:
Nephroblastoma (Wilms’ tumor – WT) is the most common solid tumor of kidney in children. We present treatment development of WT at the Department of Pediatric Hematology and Oncology, Charles University in Prague, 2^nd Faculty of Medicine and University Hospital Motol (KDHO) in the Czech Republic over 30 years. Patients that were treated prior to access to the International Society of Pediatric Oncology (SIOP) protocols are considered to be the historical group, then we have patients treated according to SIOP 9, SIOP 93–01 and SIOP 2001 protocols as full participants of SIOP studies.

Patients and Methods:
Between January 1980 and April 2009, we treated 330 patients with WT at KDHO: 91 patients in historical group (1980–1988), 94 pts in SIOP 9 (1988–1993), 80 pts in SIOP 93–01 (1994–2001) and 65 pts in SIOP 2001 (2002–2009). Overall survival (OS) and event-free survival (EFS) were analyzed by Kaplan-Meier test.

Results:
The overall ten-year EFS was 81.2% and OS 87.6%. Fifty-eight patients from the 330 (17.6%) had metastases at diagnosis, EFS without metastatic process was 84.6% compared to 65.4% with metastasis presented at diagnosis (p = 0.0003), OS was 70.7% compared to 91.2% (p < 0.0001). One hundred and seventy patients (51.5%) were treated with preoperative chemotherapy and/or radiotherapy, whereas 158 patients (47.5%) underwent primary nephrectomy; EFS and OS did not differ: neoadjuvant vs primary nephrectomy EFS was 81.2% vs 80.9% (p = 0.85), OS 89.4% vs 85.4% (p = 0.38). Sixty (18%) patients experienced disease recurrence; OS after relapse was 33%. In the historical group, EFS and OS were 85.7% and 91.2%. In patients treated according to the SIOP 9 protocol, EFS and OS were 68.1% and 74.5%, resp. In patients treated according to SIOP 93–01, it was 83.6% and 93.7%, resp. and in patients treated according to 87 SIOP 2001, it was 7% and 95.4% (p = 0.001 and p = 0.0008), resp.

Conclusion:
WT is a well treatable disease. The aim for the future is to maintain the current very good survival while minimizing the treatment intensity.

Key words:
nephroblastoma – Wilms’ tumor – treatment – prognosis

This study was supported by project of the Czech Ministry of Health – DRO, University Hospital Motol No. 00064203.

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Submitted:
29. 3. 2013

Accepted:
23. 5. 2013

Zdroje

1. Wilms M. Die Mischgeschwulste der Niere. Leipzig: Verlag von Arthur Georgi 1899.

2. Ladd WE. Embryoma of the Kidney (Wilms‘ Tumor). Ann Surg 1938; 108(5): 885– 902.

3. Gross RE, Neuhauser EB. Treatment of mixed tumors of the kidney in childhood. Pediatrics 1950; 6(6): 843– 852.

4. Farber S, Pinkel D, Sears EM et al. Advances in chemotherapy of cancer in man. Adv Cancer Res 1956; 4: 1– 71.

5. D‘Angio GJ, Beckwith JB, Bishop HC et al. Proceedings: The national Wilms‘ tumor study: a progress report. Proc Natl Cancer Conf 1972; 7: 627– 636.

6. Boccon‑ Gibod L, Rey A, Sandstedt B et al. Complete necrosis induced by preoperative chemotherapy in Wilms tumor as an indicator of low risk: report of the international society of paediatric oncology (SIOP) nephroblastoma trial and study 9. Med Pediatr Oncol 2000; 34(3): 183– 190.

7. Weirich A, Leuschner I, Harms D et al. Clinical impact of histologic subtypes in localized non‑anaplastic nephroblastoma treated according to the trial and study SIOP‑ 9/ GPOH. Ann Oncol 2001; 12(3): 311– 319.

8. Graf N, van Tinteren H, Bergeron C et al. Characteristics and outcome of stage II and III non‑anaplastic Wilms‘ tumour treated according to the SIOP trial and study 93– 01. Eur J Cancer 2012; 48(17): 3240– 3248.

9. Owens CM, Veys PA, Pritchard J et al. Role of chest computed tomography at diagnosis in the management of Wilms‘ tumor: a study by the United Kingdom Children‘s Cancer Study Group. J Clin Oncol 2002; 20(12): 2768– 2773.

10. de Kraker J, Graf N, van Tinteren H et al. Reduction of postoperative chemotherapy in children with stage Iintermediate‑ risk and anaplastic Wilms‘ tumour (SIOP 93– 01 trial): a randomised controlled trial. Lancet 2004; 364(9441): 1229– 1235.

11. Radvanský J, Slabý K, Radvanská J et al. Pozdní následky Wilmsova tumoru a jeho léčby u pacientů léčených v letech 1980– 2001 na jediném pracovišti. Klin Onkol 2010; 23(4): 245– 255.

12. D‘Angio GJ, Breslow N, Beckwith JB et al. Treatment of Wilms‘ tumor. Results of the Third National Wilms‘ Tumor Study. Cancer 1989; 64(2): 349– 360.

13. Lemerle J, Voute PA, Tournade MF et al. Preoperative versus postoperative radiotherapy, single versus multiple courses of actinomycin D, in the treatment of Wilms‘ tumor. Preliminary results of a controlled clinical trial conducted by the International Society of Paediatric Oncology (S.I.O.P.). Cancer 1976; 38(2): 647– 654.

14. Lemerle J, Voute PA, Tournade MF et al. Effectiveness of preoperative chemotherapy in Wilms‘ tumor: results of an International Society of Paediatric Oncology (SIOP) clinical trial. J Clin Oncol 1983; 1(10): 604– 609.

15. D‘Angio GJ, Evans A, Breslow N et al. The treatment of Wilms‘ tumor: results of the Second National Wilms‘ Tumor Study. Cancer 1981; 47(9): 2302– 2311.

16. D‘Angio GJ, Evans AE, Breslow N et al. The treatment of Wilms‘ tumor: Results of the national Wilms‘ tumor study. Cancer 1976; 38(2): 633– 646.

17. Tournade MF, Com‑ Nougué C, Voûte PA et al. Results of the Sixth International Society of Pediatric Oncology Wilms‘ Tumor Trial and Study: a risk‑adapted therapeutic approach in Wilms‘ tumor. J Clin Oncol 1993; 11(16): 1014– 1023.

18. Mališ J, Radvanská J, Slabý K et al. Treatment results in patients treated from 1980 to 2004 for Wilms‘ tumour in a single centre. Klin Onkol 2010; 23(5): 332– 342.

19. Green DM, Breslow NE, Beckwith JB et al. Effect of duration of treatment on treatment outcome and cost of treatment for Wilms‘ tumor: a report from the National Wilms‘ Tumor Study Group. J Clin Oncol 1998; 16(12): 3744– 3751.

20. Verschuur AC, Vujanic GM, Van Tinteren H et al. Stromal and epithelial predominant Wilms tumours have an excellent outcome: the SIOP 93 01 experience. Pediatr Blood Cancer 2010; 55(2): 233– 238.

21. Zoeller G, Pekrun A, Lakomek M et al. Wilms tumor: the problem of diagnostic accuracy in children undergoing preoperative chemotherapy without histological tumor verification. J Urol 1994; 151(1): 169– 171.

22. Grundy PE, Breslow NE, Li S et al. Loss of heterozygosity for chromosomes 1p and 16q is an adverse prognostic factor in favorable‑ histology Wilms tumor: a report from the National Wilms Tumor Study Group. J Clin Oncol 2005; 23(29): 7312– 7321.

23. Vujanić GM, Kelsey A, Mitchell C et al. The role of biopsy in the diagnosis of renal tumors of childhood: Results of the UKCCSG Wilms tumor study 3. Med Pediatr Oncol 2003; 40(1): 18– 22.

24. D‘Angio GJ. The National Wilms Tumor Study: a 40 year perspective. Lifetime Data Anal 2007; 13(4): 463– 470.

25. Vujanić GM, Sandstedt B, Harms D et al. Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood. Med Pediatr Oncol 2002; 38(2): 79– 82.