Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT00945009
Recruitment Status : Active, not recruiting
First Posted : July 23, 2009
Last Update Posted : December 4, 2017
National Cancer Institute (NCI)
Information provided by (Responsible Party):
Children's Oncology Group

Brief Summary:
This phase III clinical trial is studying how well combination chemotherapy and surgery work in treating young patients with Bilateral Wilms tumor and children who are a special risk for forming tumors in both kidneys. Drugs used in chemotherapy work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. 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.

Condition or disease Intervention/treatment Phase
Adult Renal Wilms Tumor Beckwith-Wiedemann Syndrome Childhood Renal Wilms Tumor Diffuse Hyperplastic Perilobar Nephroblastomatosis Hemihypertrophy Stage I Renal Wilms Tumor Stage II Renal Wilms Tumor Stage III Renal Wilms Tumor Stage IV Renal Wilms Tumor Stage V Renal Wilms Tumor Biological: Dactinomycin Drug: Doxorubicin Hydrochloride Drug: Vincristine Sulfate Drug: Carboplatin Drug: Cyclophosphamide Drug: Etoposide Phosphate Radiation: Radiation Therapy Procedure: Therapeutic Conventional Surgery Procedure: Adjuvant Therapy Procedure: Neoadjuvant Therapy Phase 3

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Study Type : Interventional  (Clinical Trial)
Actual Enrollment : 249 participants
Intervention Model: Single Group Assignment
Masking: None (Open Label)
Primary Purpose: Treatment
Official Title: Treatment for Patients With Bilateral, Multicentric, or Bilaterally-Predisposed Unilateral Wilms Tumor
Study Start Date : July 2009
Estimated Primary Completion Date : October 2018
Estimated Study Completion Date : October 2018

Arm Intervention/treatment
Experimental: Treatment (chemotherapy, radiation therapy, surgery)
See Detailed Description
Biological: Dactinomycin
Given IV
Other Name: Lyovac Cosmegen

Drug: Doxorubicin Hydrochloride
Given IV

Drug: Vincristine Sulfate
Given IV
Other Names:
  • Kyocristine
  • Oncovin
  • VCR
  • Vincasar

Drug: Carboplatin
Given IV

Drug: Cyclophosphamide
Given IV

Drug: Etoposide Phosphate
Given IV
Other Names:
  • ETOP
  • Etopophos

Radiation: Radiation Therapy
Undergo radiation therapy
Other Names:
  • Cancer Radiotherapy
  • Irradiate
  • Irradiated
  • Irradiation
  • RT

Procedure: Therapeutic Conventional Surgery
Undergo surgical resection

Procedure: Adjuvant Therapy
Undergo adjuvant therapy

Procedure: Neoadjuvant Therapy
Undergo neoadjuvant therapy
Other Names:
  • Induction Therapy
  • Neoadjuvant
  • Preoperative Therapy

Primary Outcome Measures :
  1. Event-free survival [ Time Frame: 4 years ]
  2. Prevention of complete removal of at least one kidney in 50% of patients with bilateral Wilms tumor (BWT) [ Time Frame: Up to 12 weeks ]
  3. Efficacy of chemotherapy in preserving renal units and preventing Wilms tumor development in patients with diffuse hyperplastic perilobular nephrogenic rests [ Time Frame: Up to 10 years ]
  4. Facilitation of partial nephrectomy in lieu of total nephrectomy in 25% of patients with unilateral Wilms tumor [ Time Frame: Up to 12 weeks ]
  5. Percentage of patients with bilateral Wilms tumor (BWT) undergoing definitive surgical treatment by week 12 [ Time Frame: 12 weeks ]

Information from the National Library of Medicine

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Ages Eligible for Study:   up to 29 Years   (Child, Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No

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)
    • 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
  • 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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT00945009

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Sponsors and Collaborators
Children's Oncology Group
National Cancer Institute (NCI)
Principal Investigator: Peter Ehrlich, MD MSC Children's Oncology Group

Responsible Party: Children's Oncology Group Identifier: NCT00945009     History of Changes
Other Study ID Numbers: AREN0534
NCI-2011-01953 ( Registry Identifier: CTRP (Clinical Trial Reporting Program) )
CDR0000649716 ( Other Identifier: Clinical )
AREN0534 ( Other Identifier: Children's Oncology Group )
AREN0534 ( Other Identifier: CTEP )
U10CA180886 ( U.S. NIH Grant/Contract )
U10CA098543 ( U.S. NIH Grant/Contract )
First Posted: July 23, 2009    Key Record Dates
Last Update Posted: December 4, 2017
Last Verified: November 2016

Additional relevant MeSH terms:
Wilms Tumor
Beckwith-Wiedemann Syndrome
Neoplasms, Complex and Mixed
Neoplasms by Histologic Type
Kidney Neoplasms
Urologic Neoplasms
Urogenital Neoplasms
Neoplasms by Site
Neoplastic Syndromes, Hereditary
Kidney Diseases
Urologic Diseases
Genetic Diseases, Inborn
Pathologic Processes
Abnormalities, Multiple
Congenital Abnormalities
Chromosome Disorders
Liposomal doxorubicin
Etoposide phosphate
Immunosuppressive Agents
Immunologic Factors
Physiological Effects of Drugs
Antirheumatic Agents