Ref.
Trial
Population
Pts (n)
Treatment
Survival outcomes
Standard risk, localized Standard risk, localized Standard risk, localized Standard risk, localized Standard risk, localized Standard risk, localized
Paulussen49
EICESS-92
Localized, Tumor volume <100ml
155
Induction (VAIA x4) + Randomization: VAIA x10 vs. VACA x10 (cyclophosphamide vs ifosfamide) 3y EFS 74% vs. 73%, HRs for EFS and overall survival 0.91 VAIA vs. VACA
Le Deley50
Euro-Ewing99 R1
<50yo Localized, either good histologic response (>90%) or Tumor volume (<200ml)
856
Induction (VIDE x6, VAI x1) Randomization: VAIx7 vs. VACx7 3y EFS and overall survival for VAI vs. VAC, 78.2% vs. 75.4% and 85.5% vs. 85.9%
Localized Localized Localized Localized Localized Localized
Grier48
INT-0091 (CCG-7881 and POG-8850)
<30yo
398
Standard (VACA) vs experimental (VACA + IE)
5yr EFS and overall survival for standard vs. experimental, 54% vs. 69% (p 0.005) and 61% vs. 72% (p 0.01)
Granowetter163
INT-0154
<30yo Localized, bone + soft tissue
478
VDC/IE (17 cycles, 48 weeks) vs. dose intensified VDC/IE (11 cycles, 30 weeks)
5y EFS and overall survival for standard vs. dose intensified, 72.1% vs. 70.1% and 80.5% vs. 77%
Womer52
COG AEWS0031
<50yr age Localized
568
Randomization: VDC/IE standard (q3/52) vs. VDC/IE intensified (q2/52)
3y EFS and overall survival for std vs. intensified, 65% vs. 73% (p 0.048) and 77% vs. 83% (p 0.056) Similar toxicity
High risk, localized* High risk, localized* High risk, localized* High risk, localized* High risk, localized* High risk, localized*
Whelan105
Euro-Ewing99/ Ewing-2008
<50yo Poor histologic response (≤90%), Tumor volume ≥200ml
240
Induction (VIDEx6, VAIx1) Randomization: VAI vs. Bu-Mel/ ASCT
8y EFS and overall survival for VAI vs. Bu-Mel, 47.1% vs. 60.7% (P 0.026) and 55.6% vs. 64.5% (p 0.028)
Metastatic (lungs only) Metastatic (lungs only) Metastatic (lungs only) Metastatic (lungs only) Metastatic (lungs only) Metastatic (lungs only)
Dirksen106
Euro-Ewing99 R2Pulm/ EWING-2008
<50yo Pulmonary/pleural metastases, nil other
287
VAI + WLI vs. Bu-Mel 3y EFS 50.6% vs. 56.6%, HR= 0.79, p=0.16 3yr OS 68% vs. 68.2%, HR=1.00, p=0.99
Multisite-metastatic (other) Multisite-metastatic (other) Multisite-metastatic (other) Multisite-metastatic (other) Multisite-metastatic (other) Multisite-metastatic (other)
Paulussen49
EICESS-92
Volume ≥100ml or ±Metastases (any)
492
VAIA x14 vs. EVAIA x4 + EVAIAx10 (addition of etoposide)
3y EFS 47% vs. 52% (p=0.47)
Brennan54
Euro-Ewing-2012
<50yo Localized +/- Metastases (lung or other)
640
VIDE/ VAI vs. VDC/ IE
HRs 0.70 for EFS, 0.64 for overall survival in favor of VDC/ IE