Purpose

This phase III trial is studying how well combination chemotherapy with or without radiation therapy works in treating young patients with newly diagnosed stage III or stage IV Wilms' tumor. 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. Radiation therapy uses high-energy x-rays to kill tumor cells. Giving more than one drug (combination chemotherapy) with or without radiation therapy may kill more tumor cells.

Conditions

Eligibility

Eligible Ages
Under 29 Years
Eligible Sex
All
Accepts Healthy Volunteers
No

Criteria

Inclusion Criteria:

- Patients must be less than 30 years of age at the time of diagnosis

- Prior to enrollment on AREN0533, all patients must have been enrolled on AREN03B2
for central pathology review. Stage III patients with LOH transferring from AREN0532
may be enrolled on this study. Eligible patients for AREN0533 must be:

- Newly diagnosed Stage IV favorable histology Wilms tumor with or without LOH 1p
and 16q or

- Newly diagnosed Stage III favorable histology Wilms tumor with LOH for 1p and
16q transferring from AREN0532

- Patients with bilateral Wilms tumors (Stage V) are not eligible for AREN0533 and
should be directed to AREN0534

- Patients must begin protocol therapy on AREN0533 by Day 14 after surgery or biopsy,
unless medically contraindicated

- The Karnofsky performance status must be >= 50 for patients > 16 years of age and
the Lansky performance status must be >= 50 for patients =< 16 years of age

- Patients cannot have had prior tumor-directed chemotherapy or radiotherapy except
for patients transferring from AREN0532 or those treated for emergent issues, as
medically indicated

- Total bilirubin ≤ 1.5 times upper limit of normal (ULN) for age (must be submitted
for central review by Day 7)

- Serum glutamic oxaloacetic transaminase (SGOT) aspartate aminotransferase (AST) or
serum glutamic pyruvic transaminase (SGPT) alanine aminotransferase (ALT) < 2.5 x
ULN for age (must be submitted for central review by Day 7)

- Shortening fraction ≥ 27% by echocardiogram OR ejection fraction ≥ 50% by
radionuclide angiogram

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

- Female patients who are lactating must agree to stop breast-feeding

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

Study Design

Phase
Phase 3
Study Type
Interventional
Allocation
N/A
Intervention Model
Single Group Assignment
Primary Purpose
Treatment
Masking
None (Open Label)

Arm Groups

ArmDescriptionAssigned Intervention
Experimental
Treatment (chemotherapy, surgery, radiotherapy)
REGIMEN DD4A (weeks 1-6): Patients receive dactinomycin IV; vincristine IV; and doxorubicin hydrochloride IV. Patients with pulmonary and extra-pulmonary metastases at diagnosis undergo radiotherapy. Patients with initially unresectable or incompletely resected tumors are reevaluated at week 6, and if resectable, undergo surgery and then proceed to either regimen DD4A or regimen M. REGIMEN DD4A (weeks 7-25): Patients receive dactinomycin IV; vincristine IV; and doxorubicin hydrochloride IV. REGIMEN M (weeks 7-31): Patients receive cyclophosphamide IV; vincristine IV; and dactinomycin IV and doxorubicin hydrochloride IV. Patients with pulmonary metastases only who are SIR also undergo whole lung radiotherapy.
  • Radiation: 3-Dimensional Conformal Radiation Therapy
    Other names:
    • 3-dimensional radiation therapy
    • 3D Conformal
    • 3D CONFORMAL RADIATION THERAPY
    • 3D CRT
    • 3D radiotherapy
    • 3D-CRT
    • Conformal Therapy
    • Radiation Conformal Therapy
    • Radiation, 3D Conformal
    • Three dimensional external beam radiation therapy (procedure)
  • Procedure: Conventional Surgery
  • Drug: Cyclophosphamide
    Given IV
    Other names:
    • (-)-Cyclophosphamide
    • 2H-1,3,2-Oxazaphosphorine, 2-[bis(2-chloroethyl)amino]tetrahydro-, 2-oxide, monohydrate
    • Asta B 518
    • B 518
    • B-518
    • B518
    • Carloxan
    • Ciclofosfamida
    • Ciclofosfamide
    • Cicloxal
    • Clafen
    • Claphene
    • CP monohydrate
    • CTX
    • CYCLO-cell
    • Cycloblastin
    • Cycloblastine
    • Cyclophospham
    • Cyclophosphamid monohydrate
    • Cyclophosphamide Monohydrate
    • Cyclophosphamidum
    • Cyclophosphan
    • Cyclophosphane
    • Cyclophosphanum
    • Cyclostin
    • Cyclostine
    • Cytophosphan
    • Cytophosphane
    • Cytoxan
    • Fosfaseron
    • Frindovyx
    • Genoxal
    • Genuxal
    • Ledoxina
    • Mitoxan
    • Neosar
    • Revimmune
    • Syklofosfamid
    • WR 138719
    • WR- 138719
    • WR-138719
    • WR138719
  • Biological: Dactinomycin
    Given IV
    Other names:
    • Actinomycin A IV
    • Actinomycin C1
    • Actinomycin D
    • Actinomycin I1
    • Actinomycin IV
    • Actinomycin X 1
    • Actinomycin-[thr-val-pro-sar-meval]
    • Cosmegen
    • DACT
    • Dactinomycine
    • Lyovac Cosmegen
    • Meractinomycin
  • Drug: Doxorubicin Hydrochloride
    Given IV
    Other names:
    • 5,12-Naphthacenedione, 10-[(3-amino-2,3,6-trideoxy-alpha-L-lyxo-hexopyranosyl)oxy]-7,8, 9,10-tetrahydro-6,8,11-trihydroxy-8-(hydroxyacetyl)-1-methoxy-, hydrochloride, (8S-cis)- (9CI)
    • ADM
    • Adriacin
    • Adriamycin
    • Adriamycin Hydrochloride
    • Adriamycin PFS
    • Adriamycin RDF
    • ADRIAMYCIN, HYDROCHLORIDE
    • Adriamycine
    • Adriblastina
    • Adriblastine
    • Adrimedac
    • Chloridrato de Doxorrubicina
    • DOX
    • DOXO-CELL
    • Doxolem
    • Doxorubicin HCl
    • Doxorubicin.HCl
    • Doxorubin
    • Farmiblastina
    • FI 106
    • FI-106
    • FI106
    • hydroxydaunorubicin
    • Rubex
  • Drug: Etoposide
    Given IV
    Other names:
    • Demethyl Epipodophyllotoxin Ethylidine Glucoside
    • EPEG
    • Lastet
    • Toposar
    • Vepesid
    • VP 16
    • VP 16-213
    • VP 16213
    • VP-16
    • VP-16-213
    • VP-16213
    • VP16
    • VP16213
  • Drug: Vincristine Sulfate Liposome
    Given IV
    Other names:
    • Marqibo

More Details

Status
Completed
Sponsor
Children's Oncology Group

Study Contact

Detailed Description

PRIMARY OBJECTIVES: I. To demonstrate that patients with Stage IV favorable histology (FH) Wilms tumor with pulmonary metastases only, who have complete resolution of the pulmonary lesions after 6 weeks of DD-4A chemotherapy (vincristine, dactinomycin, and doxorubicin), called Rapid Complete Responders (RCR), will have at least an 85% 4 year event-free survival (EFS) after therapy with additional DD-4A and without whole lung irradiation. II. To demonstrate that Stage IV FH patients who do not have resolution of pulmonary metastases by Week 6, called Slow Incomplete Responders (SIR), will have a 4 year EFS of 85% with the addition of cyclophosphamide and etoposide to a modified Regimen DD-4A (Regimen M). III. To improve the 4 year EFS to 75% for patients with Stage III or IV FH Wilms tumor with loss of heterozygosity (LOH) for chromosomes 1p and 16q. SECONDARY OBJECTIVE: I. To determine the relationship between the burden of pulmonary metastatic disease and outcome in Stage IV FH patients. OUTLINE: This is a multicenter study. REGIMEN DD4A (weeks 1-6): Patients receive dactinomycin IV over 1-5 minutes once in week 1; vincristine IV once in weeks 1-6; and doxorubicin hydrochloride IV over 15 minutes once in week 4 in the absence of disease progression or unacceptable toxicity. Patients with pulmonary and extra-pulmonary metastases at diagnosis undergo radiotherapy once daily beginning in week 1 and continuing for 5-14 days. After completion of DD4A chemotherapy (week 6), patients undergo evaluation. Patients with stage IV disease and pulmonary metastases only with no loss of heterozygosity (LOH) who are rapid complete responders (RCR) (i.e., pulmonary metastases disappear) proceed to regimen DD4A (weeks 7-25). All other patients (i.e., patients with stage III or IV disease and LOH of both 1p and 16q; stage IV disease with pulmonary metastases only who are slow incomplete responders [SIR] [i.e., pulmonary metastases do not disappear]; or stage IV disease with nonpulmonary metastases or with nonpulmonary metastases in combination with pulmonary metastases) proceed to regimen M (weeks 7-31). Patients with initially unresectable or incompletely resected tumors are reevaluated at week 6, and if resectable, undergo surgery and then proceed to either regimen DD4A or regimen M as described above. REGIMEN DD4A (weeks 7-25): Patients receive dactinomycin IV over 1-5 minutes once in weeks 7, 13, 19, and 25; vincristine IV once in weeks 7-10, 13, 16, 19, 22, and 25; and doxorubicin hydrochloride IV over 15 minutes once in weeks 10, 16, and 22 in the absence of disease progression or unacceptable toxicity. REGIMEN M (weeks 7-31): Patients receive cyclophosphamide IV over 1 hour and etoposide IV over 1 hour on days 1-5 in weeks 7, 10, 19, and 25; vincristine IV once in weeks 8, 9, 11, 12, 13, 16, 22, 28, and 31; and dactinomycin IV and doxorubicin hydrochloride IV over 15 minutes once in weeks 13, 16, 22, 28, and 31 in the absence of disease progression or unacceptable toxicity. Patients with pulmonary metastases only who are SIR also undergo whole lung radiotherapy once daily beginning in week 7 and continuing for 5-14 days. NOTE: Patients who begin study treatment after undergoing resection of pulmonary metastases are treated according to regimen DD4A (weeks 1-25) and undergo whole lung radiotherapy for 5-14 days beginning in week 1. After completion of study treatment, patients are followed periodically for 10 years.

Notice

Study information shown on this site is derived from ClinicalTrials.gov (a public registry operated by the National Institutes of Health). The listing of studies provided is not certain to be all studies for which you might be eligible. Furthermore, study eligibility requirements can be difficult to understand and may change over time, so it is wise to speak with your medical care provider and individual research study teams when making decisions related to participation.