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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Wilms Tumor

Standard Treatment Options

Table 2 describes the standard chemotherapy regimens used to treat Wilms tumor.

Table 2. Standard Chemotherapy Regimens for Wilms Tumor

Regimen NameRegimen Description
Regimen EE-4A[1]Vincristine, dactinomycin x 18 weeks postnephrectomy
Regimen DD-4A[1]Vincristine, dactinomycin, doxorubicin x 24 weeks postnephrectomy
Regimen I[2]Vincristine, doxorubicin, cyclophosphamide, etoposide x 24 weeks

Table 3 provides an overview of the standard treatment based on published results for all stages of Wilms tumor and survival information.

Table 3. Overview of Wilms Tumor Standard Treatment by Stage

StageHistology4 Year RFS or EFS4 Year OSTreatment (seeTable 2for chemotherapy regimen descriptions)
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
a Abdominal XRT is planned according to local stage of renal tumor.
b Pulmonary XRT is reserved for patients with chest x-ray evidence of pulmonary metastases.
c 90% of the relapses occurred by 3.8 years from diagnosis and 90% of the deaths occurred within 5.7 years from diagnosis.[3]
d This approach is changing as noted on the AREN0534 study.
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[1,2,3]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 XRTd
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