Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor

Inclusion Criteria:

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

      - Synchronous bilateral Wilms tumors**; or

      - 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

      - Multicentric Wilms tumor (any age) (to be eligible, these patients must not
      undergo any nephrectomy at diagnosis); or

      - 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

      - Diffuse hyperplastic perilobar nephroblastomatosis (unilateral or bilateral)
      defined by central radiological review; or

      - Wilms tumor arising in a solitary kidney (patients with metachronous Wilms tumor
      are not eligible)

         - 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

   - Specimens/materials must 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

   - Patients must begin protocol therapy on AREN0534 by day 14 following surgery or
   diagnosis by initial computed tomography (CT)/magnetic resonance imaging (MRI), unless
   medically contraindicated

   - Karnofsky performance status must be >= 50% for patients > 16 years of age and 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

   - Patients with unilateral Wilms tumor and aniridia, Beckwith-Wiedemann Syndrome,
   idiopathic hemihypertrophy, Simpson-Golabi-Behmel-Syndrome, Denys-Drash Syndrome or
   other associated genitourinary anomalies; or multicentric or unilateral Wilms tumor
   with contralateral nephrogenic rest(s) (any size) in a child under 1 year of age who
   undergo a nephrectomy at diagnosis are not eligible for this study and should be
   directed to a unilateral Wilms tumor study

   - Total bilirubin =< 1.5 times upper limit of normal (ULN) for age

   - Serum glutamic oxaloacetic transaminase (SGOT) (aspartate aminotransferase [AST]) or
   serum glutamate pyruvate transaminase (SGPT) (alanine aminotransferase [ALT]) < 2.5
   times upper limit of normal (ULN) for age

   - Shortening fraction >= 27% by echocardiogram, 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])

   - Female patients of childbearing age must have a negative pregnancy test

   - Female patients who are lactating must agree to stop breastfeeding

   - Sexually active patients of childbearing potential must agree to use effective
   contraception

   - All patients and/or their parents or legal guardians must sign a written informed
   consent

   - All institutional, Food and Drug Administration (FDA), and National Cancer Institute
   (NCI) requirements for human studies must be met

   - No concurrent aprepitant