HEALTH PROFESSIONAL · SOURCE READING
Treatment of stage IV diffuse anaplastic Wilms tumor
Source: Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 15, 2025 · Captured 2026-09-09.
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Context: Wilms Tumor / Treatment of Wilms Tumor / Treatment of stage IV Wilms tumor
In the AREN0321 (NCT00335556) study, the combination of vincristine and irinotecan (VI) was tested in an up-front window for patients with diffuse anaplastic Wilms tumor and measurable disease.[267][Level of evidence C2]
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Fourteen patients with stage IV diffuse anaplastic Wilms tumor with measurable disease received the window therapy; one patient achieved a complete response (CR), ten patients achieved partial responses (PRs), and no patients had stable disease. This resulted in a CR and PR rate of 79%.
Patients who responded to VI in the window therapy had VI incorporated into their regimen (UH2).
Because of the observed cardiac/pulmonary toxicities encountered in this trial, the study was interrupted and amended with reduced doses of doxorubicin, cyclophosphamide, and etoposide (when combined with carboplatin).
Further study of the modified regimen is planned in patients with newly diagnosed diffuse anaplastic Wilms tumor.
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