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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)

  • Updated: 03/27/2014

Table 3. Overview of Wilms Tumor Standard Treatment by Stage

Stage Histology 4 Year RFS or EFS 4 Year OS Treatment (see Table 2 for chemotherapy regimen descriptions) 
Stage I [1,2,4]FH <24 mo/tumor weight <550g85%98%Surgery only (should be done only within the context of a clinical trial)
FH >24 mo/tumor weight >550g94% RFS98%Nephrectomy + lymph node sampling followed by regimen EE-4A
DA68% EFS79%; (n = 10)Nephrectomy + lymph node sampling followed by regimen EE-4A and XRT
Stage II [1,2]FH86% RFS98%Nephrectomy + lymph node sampling followed by regimen EE-4A
FA80% EFS80%; (n = 5)Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A
DA83% EFS82%Nephrectomy + lymph node sampling followed by abdominal XRT and regimen I
Stage III [1,2]FH87% RFS94%Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A
FA88% RFS100%; (n = 8)Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A
FA (preoperative treatment)71% RFS71%; (n = 7)Preoperative treatment with regimen DD-4A followed by nephrectomy + lymph node sampling and abdominal XRT
DA46% EFS53%; (n = 16)Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling and abdominal XRT
DA65% EFS67%Immediate nephrectomy + lymph node sampling followed by abdominal XRT and regimen I
Stage IV [1,2]FH76% RFS86%Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A
FA61% EFS72%; (n = 11)Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A
DA33% EFS33%; (n = 15)Immediate nephrectomy + lymph node sampling followed by abdominal XRT,a whole-lung XRT,b and regimen I
DA (preoperative treatment)31% EFS44%; (n = 13)Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling, followed by abdominal XRT,a and whole-lung XRTb
Stage V [1-3]Overall61% EFS80%; (n = 158)
FH65%87% (4-yr OS); 78% (10-yr OS)cBilateral 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 XRTd
FA76%88%; (n = 9)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 XRTd
DA25%42%; (n = 20)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 d

AH = anaplastic histology; DA = diffuse anaplastic; EFS = event-free survival; FA = focal anaplastic; FH = favorable histology; OS = overall survival; RFS = relapse-free survival; XRT = 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.
c90% of the relapses occurred by 3.8 years from diagnosis and 90% of the deaths occurred within 5.7 years from diagnosis.[3]
dThis approach is changing as noted on the AREN0534 study.

References

  1. 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 23 (29): 7312-21, 2005.  [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. Ehrlich PF: Bilateral Wilms' tumor: the need to improve outcomes. Expert Rev Anticancer Ther 9 (7): 963-73, 2009.  [PUBMED Abstract]

  4. Shamberger RC, Anderson JR, Breslow NE, et al.: Long-term outcomes for infants with very low risk Wilms tumor treated with surgery alone in National Wilms Tumor Study-5. Ann Surg 251 (3): 555-8, 2010.  [PUBMED Abstract]