Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor
Condition: Adult Kidney Wilms Tumor; Beckwith-Wiedemann Syndrome; Childhood Kidney Wilms Tumor; Diffuse Hyperplastic Perilobar Nephroblastomatosis; Rhabdoid Tumor of the Kidney; Stage I Kidney Wilms Tumor; Stage II Kidney Wilms Tumor; Stage III Kidney Wilms Tumor; Stage IV Kidney Wilms Tumor; Stage V Kidney Wilms Tumor
MeSH terms: Neoplasms; Wilms Tumor; Rhabdoid Tumor; Kidney Neoplasms; Beckwith-Wiedemann Syndrome
Intervention: Dactinomycin; Doxorubicin Hydrochloride; Radiation Therapy; Therapeutic Conventional Surgery; Vincristine Sulfate
Intervention description: Given IV; Given IV; Undergo radiation therapy; Undergo surgical resection; Given IV
Intervention MeSH terms: Dactinomycin; Cactinomycin; Doxorubicin; Liposomal doxorubicin; Vincristine
Phase: Phase 3
Study type: Interventional
Primary purpose: Treatment
Allocation: Non-Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Enrollment: 249.0
Sex: All
Maximum age: 29 Years
Healthy volunteers: No

Summary:

      This phase III trial studies how well combination chemotherapy and surgery work in treating
      young patients with Wilms tumor. Drugs used in chemotherapy work in different ways to stop
      the growth of tumor cells, either by killing the cells, by stopping them from dividing, or by
      stopping them from spreading. Giving more than one drug (combination chemotherapy) may kill
      more tumor cells. Giving combination chemotherapy before surgery may make the tumor smaller
      and reduce the amount of normal tissue that needs to be removed. Giving it after surgery may
      kill any tumor cells that remain after surgery.
    

Eligibility criteria:

        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