Ewing sarcoma, pediatric

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Upfront Therapy

COG AEWS1031 Regimen A

Study	Dates of enrollment	Evidence	Comparator	Comparative Efficacy
Leavey et al. 2021 (COG AEWS1031)	2010-11-22 to 2016-01-04	Phase 3 (C)	COG AEWS1031 Regimen B	Did not meet co-primary endpoints of EFS/OS

Note: Regimen A is a standard 17-week regimen consisting of induction and continuation.

Induction

Chemotherapy, VDC portion

• Vincristine (Oncovin) as follows:
  • Cycles 1, 3, 5: 1.5 mg/m²/dose (maximum dose of 2 mg) IV push once per day on day 1 of weeks 1, 2, 5, 6, 9 & 10
    • For children < 10 kg or < 1 year, the dose is 0.05 mg/kg/dose
• Doxorubicin (Adriamycin) as follows:
  • Cycles 1, 3, 5: 37.5 mg/m²/dose IV push/infusion over 1 to 15 minutes on days 1 and 2 of weeks 1, 5 and 9.
    • For children < 10 kg or < 1 year, the dose is 1.25 mg/kg/dose
    • Administer at a concentration not to exceed 2 mg/mL. Doxorubicin (Adriamycin) should be administered through the tubing of rapidly infusing solution of D5W or 0.9% NaCl, and it should not be infused into a large vein
• Cyclophosphamide (Cytoxan) as follows:
  • Cycles 1, 3, 5: 1200 mg/m²/dose IV over 30 to 60 minutes on day 1 of weeks 1, 5 and 9
    • For children < 10 kg or < 1 year, the dose is 40 mg/kg/dose
    • May be administered as undiluted drug (20 mg/mL, reconstitute with 0.9% NaCl to avoid hypotonic solution) or further diluted

Supportive therapy, VDC portion

• Mesna (Mesnex) as follows:
  • Cycles 1, 3, 5: 720 mg/m²/day by IV infusion or IV/PO with cyclophosphamide on day 1 of weeks 1, 5 and 9
    • For children < 10 kg or < 1 year, the dose is 24 mg/kg/dose

Chemotherapy, IE portion

• Ifosfamide (Ifex) as follows:
  • Cycles 2, 4, 6: 1800 mg/m²/dose IV infusion over 1 hour on days 1, 2, 3, 4, and 5 of Weeks 3, 7 and 11
    • For children < 10 kg or < 1 year, the dose is 60 mg/kg/dose
    • Achieve urine specific gravity ≤ 1.010 before starting ifosfamide.
• Etoposide (Vepesid) as follows:
  • Cycles 2, 4, 6: 100 mg/m²/dose IV infusion over 1 to 2 hours once on days 1, 2, 3, 4, and 5 of weeks 3, 7 and 11
    • For children < 10 kg or < 1 year, the dose is 3.3 mg/kg/dose

Supportive therapy, IE portion

• Mesna (Mesnex) as follows:
  • Cycles 2, 4, 6: 1080 mg/m²/day by IV infusion or IV/PO with ifosfamide on days 1, 2, 3, 4, and 5 of Weeks 3, 7 and 11
    • For children < 10 kg or < 1 year, the dose is 36 mg/kg/dose

14-day cycle for 6 cycles, followed by:

Continuation

To be completed

References

1. COG AEWS1031: Leavey PJ, Laack NN, Krailo MD, Buxton A, Randall RL, DuBois SG, Reed DR, Grier HE, Hawkins DS, Pawel B, Nadel H, Womer RB, Letson GD, Bernstein M, Brown K, Maciej A, Chuba P, Ahmed AA, Indelicato DJ, Wang D, Marina N, Gorlick R, Janeway KA, Mascarenhas L. Phase III Trial Adding Vincristine-Topotecan-Cyclophosphamide to the Initial Treatment of Patients With Nonmetastatic Ewing Sarcoma: A Children's Oncology Group Report. J Clin Oncol. 2021 Dec 20;39(36):4029-4038. Epub 2021 Oct 15. Erratum in: J Clin Oncol. 2022 Jul 20;40(21):2393. link to original article link to PMC article PubMed Clinical Trial Registry