Trial Information

Treatment for Very Low and Standard Risk Favorable Histology Wilms Tumor

PRIMARY OBJECTIVES:

I. Evaluate the overall and event-free survival of younger patients with newly diagnosed
stage I favorable histology Wilms' tumor (< 2 years of age and < 550gms) treated with
nephrectomy only (very low risk), or with newly diagnosed stage III favorable histology
Wilms tumor with possible nephrectomy followed by vincristine, dactinomycin, doxorubicin
hydrochloride, and radiotherapy (standard risk).

SECONDARY OBJECTIVES:

I. Determine the effects of adding doxorubicin hydrochloride to the regimen for patients
with stage I or II favorable histology found to have a high-risk biological marker.

II. Determine whether the omission of adjuvant therapy increases the incidence of
contralateral kidney lesions in patients with very low-risk disease treated by nephrectomy
and observation only.

III. Determine whether the omission of adjuvant therapy increases the incidence of renal
failure in patients with very low-risk disease who have metachronous relapse.

IV. Correlate study outcomes in patients with standard-risk disease with biological data
from tissue collections on protocol study COG-AREN03B2.

OUTLINE: This is a multicenter study. Patients are stratified according to clinical and
biological risk factors (very low risk vs standard risk).

STRATUM I: (very low-risk disease) Patients undergo nephrectomy only. If they meet criteria,
they are then observed periodically for 5 years. Patients with recurrent disease undergo
surgery (immediate or delayed) and receive chemotherapy as in stratum III. Patients with no
metachronous renal disease receive radiotherapy. Patients with metachronous disease undergo
renal-sparing surgery and chemotherapy as in stratum III, but no radiotherapy. Treatment
continues for up to 25 weeks.

STRATUM II: (standard-risk, stage I or II disease with adverse biological marker) Patients
undergo nephrectomy. Between 9 and 14 days post-nephrectomy, patients receive vincristine IV
beginning on day 1, every week for 10 weeks then every 3 weeks for a total of 15 doses.
Patients receive dactinomycin IV beginning day 1, alternating every 3 weeks with doxorubicin
hydrochloride IV for a total of 5 doses of dactinomycin and 4 doses of doxorubicin.
Treatment continues for up to 25 weeks.

STRATUM III: (standard-risk, stage III disease) Patients undergo nephrectomy, if feasible,
or biopsy. For patients who undergo biopsy only, definitive surgery is undertaken at week 7
or 13. Between 9 and 14 days post-nephrectomy, patients receive vincristine IV beginning on
day 1 every week for 10 weeks then every 3 weeks for a total of 15 doses. Patients receive
dactinomycin IV beginning day 1, alternating every 3 weeks with doxorubicin hydrochloride IV
for a total of 5 doses of dactinomycin and 4 dose of doxorubicin hydrochloride. Patients
undergo radiotherapy over 5-7 days after nephrectomy. Treatment continues for up to 25
weeks.

After completion of study treatment, patients are followed periodically for up to 8 years.