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. Author manuscript; available in PMC: 2017 Oct 5.
Published in final edited form as: Ann Surg. 2017 Sep;266(3):470–478. doi: 10.1097/SLA.0000000000002356

Table 2B. Treatment Regimens for Bilateral Wilms Tumor at Week 6.

BWT with COMPLETE RESPONSE AFTER WEEK 6 IMAGING REVIEW
Stage Chemotherapy Regimen
CR by imaging after 6 weeks of chemotherapy, no biopsy done Localized disease at initial imaging EE4A
CR by imaging after 6 weeks of chemotherapy Metastatic disease on initial imaging DD4A
CR by imaging after 6 weeks of chemotherapy, initial biopsy performed All Stages DD-4A for favorable histology Revised UH-1 for anaplastic histology
SURGICAL RESECTION AT 6 WEEKS FURTHER CHEMOTHERAPY IS BASED ON HISTOLOGY OF REMOVED TUMOR AND HIGHEST TUMOR STAGE.
  Histology   Stage   Regimen
  Completely necrotic I-II  EE-4A
  Intermediate risk  I  EE-4A
  Intermediaterisk  II  DD-4A
  Intermediate risk III-IV DD-4A+XRT
  Blastemal predominant  I  DD-4A
  Diffuse anaplastic Wilms tumor  I DD-4A+XRT
  Completely necrotic III-IV DD-4A+XRT
  Focal anaplastic Wilms tumor I-III D-4A+XRT
  Blastemal predominant  II  Reg. I
  Blastemal predominant III-IV  Reg. I +XRT
  Focal anaplastic Wilms tumor  IV  Revised UH-1+XRT
  Diffuse anaplastic Wilms tumor II-IV  Revised UH-1)+XRT
 PARTIAL RESPONSE IN BOTH KIDNEYS BUT BILATERAL PARTIAL NEPHRECTOMY NOT FEASIBLE AT 6 WEEKS. NO SURGERY SHOULD BE PERFORMED. CONTINUE CHEMOTHERAPY.
  Histology   Stage   Regimen
 If initial therapy based on imaging only All stages 6 more weeks of VAD Reevaluate at 12 weeks.
 If initial therapy based on imaging and biopsy revealed favorable histology All stages  6 more weeks of VAD
 If initial therapy based on imaging and biopsy revealed anaplastic Wilms tumor All stages Continue with Revised UH-1 Reevaluate at 12 weeks.
 LESS THAN PARTIAL RESPONSE IN EITHER KIDNEY AT 6 WEEKS. THESE PATIENTS REQUIRE BILATERAL OPEN BIOPSIES.
 6-week biopsy- Reveals anaplasia   All stages  Revised Regimen UH-1 and treat per stage. Reassess at 12 weeks.
 6-week biopsy- Reveals blastemal predominant   All stages   Regimen I. Reassess at 12 weeks.
 6-week biopsy- All other histology   All stages Continue VAD for 6 weeks and reassess at 12 weeks.

CR= Complete Response

XRT= Radiation therapy

VAD= Vincristine, dactinomycin and doxorubicin

EE4A= Vincristine and dactinomycin I= Vincristine, dactinomycin, doxorubicin, cyclophosphamide and etoposide

UH-1 = Vincristine, dactinomycin doxorubicin cyclophosphamide carboplatin and etoposide