Brief 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.

Trial Health

On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment

249

participants targeted

Target at P50-P75 for phase_3

Geographic Reach

6 countries

169 active sites

Status

active not recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

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Study Timeline

Key milestones and dates

Study Start

First participant enrolled

July 13, 2009

Completed

9 days until next milestone

First Submitted

Initial submission to the registry

July 22, 2009

Completed

1 day until next milestone

First Posted

Study publicly available on registry

July 23, 2009

Completed

7.4 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

December 31, 2016

Completed

2.2 years until next milestone

Results Posted

Study results publicly available

March 5, 2019

Completed

Last Updated

May 31, 2025

Status Verified

May 1, 2025

Enrollment Period

7.5 years

First QC Date

July 22, 2009

Results QC Date

November 5, 2018

Last Update Submit

May 22, 2025

Conditions

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

Outcome Measures

Primary Outcomes (5)

Event-Free Survival (EFS)
Probability of no relapse, secondary malignancy, or death whichever occurs first
4 years from study enrollment
Kidney Preservation After Preoperative Chemotherapy
Prevention of complete removal of at least one kidney in 50% of patients with bilateral Wilms tumor (BWT).
12 weeks from study entry
Number of Patients Without Complete Removal of at Least One Kidney
To evaluate the efficacy of chemotherapy in preserving renal units in children with diffuse hyperplastic perilobar nephroblastomatosis (DHPLN) and preventing Wilms tumor development.
12 weeks from the study entry
Percentage of Patients Who Experienced Partial Nephrectomy After Preoperative Chemotherapy
Percentage of patients who experienced partial nephrectomy in lieu of nephrectomy in 25% of children with unilateral tumors and aniridia, Beckwith-Wiedemann syndrome (BWS), hemihypertrophy or other overgrowth syndromes, by using prenephrectomy 2-drug chemotherapy induction with vincristine and dactinomycin.
12 weeks from study entry
Percentage of Patients Who Had Definitive Surgical Treatment
Percentage of Bilateral Wilms Tumor (BWT) patients who undergo definitive surgery by week 12 after initiation of chemotherapy.
12 weeks from study entry

Study Arms (3)

Arm 1 (Bilateral Wilms Tumors)

EXPERIMENTAL

Patients start with three drug chemotherapy (Regimen VAD; vincristine, dactinomycin and doxorubicin) and are evaluated and six and 12 weeks for feasibility of undergoing a partial nephrectomy/renal sparing surgery. At week 12 definitive surgery takes place followed by chemotherapy and radiation therapy based on histology and stage. Treatment continues for 25 or 31 weeks depending on histology. Patients are followed for up to 10 years following end of therapy.

Biological: DactinomycinDrug: Doxorubicin HydrochlorideRadiation: Radiation TherapyProcedure: Therapeutic Conventional SurgeryDrug: Vincristine Sulfate

Arm 2 (Unilateral High Risk tumors bilaterally predisposed)

EXPERIMENTAL

Patients start with either 2 drug or three drug chemotherapy (Regimen VA, VAD) and are evaluated a 6 and 12 weeks for feasibility of undergoing a partial nephrectomy. At week 12 definitive surgery takes place followed by chemotherapy.

Biological: DactinomycinDrug: Doxorubicin HydrochlorideRadiation: Radiation TherapyProcedure: Therapeutic Conventional SurgeryDrug: Vincristine Sulfate

Arm 3 (DHPLN)

EXPERIMENTAL

Patients with this rare disease are diagnosed based on cross-sectional imaging characteristics and undergo 2 drug chemotherapy (Regimen;VA). Patients are reassessed at 6 weeks and 12 weeks. If disease has responded or stayed stable chemotherapy is completed for 19 weeks (Regimen EE4A). If disease has progress a biopsy is performed to assess histology and adjust therapy based on the biopsy. This therapy may include, nephrectomy, chemotherapy or radiation therapy.

Biological: DactinomycinDrug: Doxorubicin HydrochlorideRadiation: Radiation TherapyProcedure: Therapeutic Conventional SurgeryDrug: Vincristine Sulfate

Interventions

DactinomycinBIOLOGICAL

Given IV

Also known as: 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