Title (COG AREN0534) Treatment for Patients with Bilateral, Multicentric, or Bilaterally-Predisposed Unilateral Wilms Tumor
Principal Investigator Martin Brecher, MD
Study Number 168709
Summary Children with bilateral Wilms tumor account for 5-7% of all patients with Wilms tumor. Certain patients, with syndromes associated with Wilms tumor development, have been identified to be at increased risk for bilateral tumors. Due to an increased risk for renal failure, patients with bilateral disease at presentation are treated with preoperative chemotherapy in order to preserve renal parenchyma. Although this recommendation was made nearly 30 years ago, patients with bilateral tumors have not been formally studied in prior cooperative trials. Recent evidence suggests that survival of these patients is inferior to similar patients with unilateral tumor. This study is designed to improve the survival of these children, while continuing the emphasis on preserving renal function.
- Enrollment on the AREN03B2 Renal Tumor Biology and Classification Study is required prior to enrollment on AREN0532.
- Patients must be < 30 years old at the time of initial diagnosis.
- The patient must have one of the following conditions to be eligible for AREN0534: a)Synchronous or metachronous bilateral Wilms tumors; or b) Unilateral Wilms tumor and aniridia, Becwith-Wiedemann Syndrome, idiopathic hemihypertrophy, Simpson-Golabi-Behmel-Syndrome, Denys-Drash Syndrome or other associated genitourinary anomalies; or c) Multicentric Wilms tumor or unilateral WT with contralateral nephrogenic rest(s) in a child under one year of age; or d) Diffuse hyperplastic perilobar nephroblastomatosis; or e) Wilms tumor arising in a solitary kidney.
- Specimens/materials per Section 5.1 of AREN03B2 should be submitted for central review by Day 7. For enrollment on AREN0534, unless a biopsy was done, the submission requirements at enrollment on AREN03B2 refer to imaging studies. Tissue samples are only required if a surgical procedure (biopsy or nephrectomy) was performed at the time of enrollment on AREN03B2.
- The Karnofsky performance status must be >= 50 for patients > 16 years of age and the Lansky performance status must be >= 50 for patients <= 16 years of age.
- Patients must not have received systemic chemotherapy or radiation therapy prior to treatment on this study. the only exception is for patients with metachronous Wilms tumor developing in a solitary kidney after previous Wilms tumor treatment.
- Adequate liver and cardiac functions defined in section 3.2.6 of the protocol.
- Female patients of childbearing age must have a negative pregnancy test. Female patients who are lactating must agree to stop breast-feeding. Sexually active patients of childbearing potential must agree to use effective contraception.