Clinical Trials

Study closed to enrollment

Inclusion criteria:

Patients must be < 30 years old at the time of initial diagnosis. Patients must be previously enrolled on AREN03B2 and confirmed to be eligible for AREN0534.

The patient must have one of the following conditions to be eligible for AREN0534:

1. Synchronous bilateral Wilms tumors*; or
2. Unilateral Wilms tumor and aniridia, Beckwith-Wiedemann Syndrome, idiopathic hemihypertrophy, Simpson-Golabi-Behmel-Syndrome, Denys-Drash Syndrome or other associated genitourinary anomalies associated with bilateral Wilms tumor, such as hypospadias and undescended testis (to be eligible, these patients must not undergo any nephrectomy at diagnosis; Note-horseshoe kidney is not associated with bilateral Wilms tumor and these patients should go on the appropriate unilateral Wilms tumor study); or
3. Multicentric Wilms tumor (any age) (to be eligible, these patients must not undergo any nephrectomy at diagnosis); or
4. Unilateral Wilms tumor with contralateral nephrogenic rest(s) (any size) in a child under one year of age (to be eligible, these patients must not undergo any nephrectomy at diagnosis); or
5. Diffuse hyperplastic perilobar nephroblastomatosis (unilateral or bilateral) defined by central radiological review; or
6. Wilms tumor arising in a solitary kidney (patients with metachronous Wilms tumor are not eligible).

(*) It is often difficult to distinguish Wilms tumors from nephrogenic rests based on imaging studies and percutaneous biopsies. The AREN0534 study uses the guideline that Wilms tumor with a single lesion 1 cm or greater in the contralateral kidney or multiple lesions (of any size) in the contralateral kidney should be treated on the synchronous bilateral Wilms tumor stratum. Patients with an isolated lesion less than 1 cm in the contralateral kidney should be treated on the appropriate study for unilateral Wilms tumor OR on the unilateral Wilms tumor/contralateral nephrogenic rest stratum of this study if they have not undergone nephrectomy and are under one year of age.

Loss of heterozygosity (LOH) results which are used in the unilateral Wilms tumor studies are not a requirement for enrollment on AREN0534. Blood samples can be submitted but will not be used to direct AREN0534 therapy.