Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor

DISEASE CHARACTERISTICS:

• Diagnosis of 1 of the following:

  • Synchronous or metachronous bilateral Wilms tumor (BWT)*

  • Unilateral Wilms tumor (WT) 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)

  • Multicentric WT (any age)

    • To be eligible, these patients must not undergo any nephrectomy at diagnosis

  • Multicentric WT or unilateral WT with contralateral nephrogenic rest(s) (any size) in a patient < 1 year of age

    • To be eligible, these patients must not undergo any nephrectomy at diagnosis
  • Diffuse hyperplastic perilobar nephroblastomatosis (unilateral or bilateral) defined by central radiological review

  • WT arising in a solitary kidney

    • Patients with metachronous WT are not eligible NOTE: *It is often difficult to distinguish WT from nephrogenic rests based on imaging studies and percutaneous biopsies. The AREN0534 study uses the guideline that WT 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 WT stratum. Patients with an isolated lesion less than 1 cm in the contralateral kidney should be treated on the appropriate study for unilateral WT OR on the unilateral WT/contralateral nephrogenic rest stratum of this study if they have not undergone nephrectomy and are under one year of age.
• Patients with BWT who underwent total or partial nephrectomy at diagnosis are eligible
• Previously enrolled in COG-AREN03B2
• Loss of heterozygosity (LOH) results—which are used in the unilateral WT studies—are not a requirement for enrollment on AREN0534
• Patients must begin protocol therapy by Day 14 following surgery or diagnosis by initial CT/MRI, unless medically contraindicated

PATIENT CHARACTERISTICS:

• Karnofsky performance status (PS) 50-100% (for patients > 16 years of age) or Lansky PS 50-100% (for patients ≤ 16 years of age)
• Total bilirubin ≤ 1.5 times upper limit of normal (ULN) for age
• AST or ALT < 2.5 times ULN for age

• Shortening fraction ≥ 27% by ECHO OR ejection fraction ≥ 50% by radionuclide angiogram

  • Cardiac function does not need to be assessed in patients who will not receive doxorubicin as part of their initial therapy on this study (i.e., patients who start on Regimen EE-4A)
• Not pregnant or nursing
• Negative pregnancy test
• Fertile patients must use effective contraception

PRIOR CONCURRENT THERAPY:

• See Disease Characteristics
• No prior systemic chemotherapy or radiotherapy
• No concurrent aprepitant