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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)
Patient VersionHealth Professional VersionEn españolLast Modified: 11/07/2008
Table 2. Overview of Wilms Tumor Standard Treatment by Stage

Stage  Histology  RFS or EFS  OS  Treatment (see Table 1 for chemotherapy regimen definitions) 
Stage I [1-3] FH 92% RFS 98% Nephrectomy + lymph node sampling followed by regimen EE-4A
FA or DA 69% EFS 83% Nephrectomy + lymph node sampling followed by regimen EE-4A and XRT
Stage II [1,2,4] FH 85% RFS 96% Nephrectomy + lymph node sampling followed by regimen EE-4A
FA (very small numbers) 80% EFS 80% Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A
DA 83% EFS 82% Nephrectomy + lymph node sampling followed by abdominal XRT and regimen I
Stage III [1,2] FH 90% RFS 95% Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A
FA 88% RFS 100% Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A
FA 71% RFS 71% Preoperative treatment with regimen DD-4A followed by nephrectomy + lymph node sampling and abdominal XRT
DA 46% EFS 53% Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling and abdominal XRT
DA 65% EFS 67% Immediate nephrectomy + lymph node sampling followed by abdominal XRT and regimen I
Stage IV [1,2,4] FH 80% RFS 90% Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A
FA 61% EFS 72% Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A
DA 33% EFS 33% Immediate nephrectomy + lymph node sampling followed by abdominal XRT,a whole-lung XRT,a and regimen I
DA 31% EFS 44% Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling, followed by abdominal XRT,a and whole-lung XRTb
Stage V [1,5-13] FH 65% 78% (10 year OS) Bilateral renal biopsies and staging of each kidney followed by preoperative treatment with regimen EE-4A (if disease in both kidneys ≤ stage II) or regimen DD-4A (if disease in both kidneys > stage II), followed by second look surgery and possibly more chemotherapy and/ or XRT
AH 44% 55% Bilateral renal biopsies and staging of each kidney followed by preoperative treatment with regimen I, followed by second look surgery and possibly more chemotherapy and/ or XRT
AH = anaplastic histology; DA = diffuse anaplastic; EFS = event-free survival; FA = focal anaplastic; FH = favorable histology; OS = overall survival; RFS = relapse-free survival; XRT = flank radiation therapy
aAbdominal XRT is planned according to local stage of renal tumor.
bPulmonary XRT is reserved for patients with chest x-ray evidence of pulmonary metastases.

References

  1. Green DM, Breslow NE, Beckwith JB, et al.: Comparison between single-dose and divided-dose administration of dactinomycin and doxorubicin for patients with Wilms' tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol 16 (1): 237-45, 1998.  [PUBMED Abstract]

  2. Dome JS, Cotton CA, Perlman EJ, et al.: Treatment of anaplastic histology Wilms' tumor: results from the fifth National Wilms' Tumor Study. J Clin Oncol 24 (15): 2352-8, 2006.  [PUBMED Abstract]

  3. Green DM, Beckwith JB, Breslow NE, et al.: Treatment of children with stages II to IV anaplastic Wilms' tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol 12 (10): 2126-31, 1994.  [PUBMED Abstract]

  4. 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 16 (12): 3744-51, 1998.  [PUBMED Abstract]

  5. Montgomery BT, Kelalis PP, Blute ML, et al.: Extended followup of bilateral Wilms tumor: results of the National Wilms Tumor Study. J Urol 146 (2 ( Pt 2)): 514-8, 1991.  [PUBMED Abstract]

  6. Zuppan CW, Beckwith JB, Weeks DA, et al.: The effect of preoperative therapy on the histologic features of Wilms' tumor. An analysis of cases from the Third National Wilms' Tumor Study. Cancer 68 (2): 385-94, 1991.  [PUBMED Abstract]


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