Ewing sarcoma, pediatric

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This page contains studies that were specific to pediatric populations. For the more general Ewing sarcoma page, follow this link.
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Last updated on 2024-07-23:1 regimens on this page
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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 (cycles 1, 3, 5)====
*Vincristine (Oncovin) by the following age- and weight-based criteria:
**10 kg or more AND 12 months old or older: 1.5 mg/m2 (maximum dose of 2 mg) IV push once per day on days 1 & 8
**Less than 10 kg OR younger than 12 months old: 0.05 mg/kg IV push once per day on days 1 & 8
*Doxorubicin (Adriamycin) by the following age- and weight-based criteria:
**10 kg or more AND 12 months old or older: 37.5 mg/m2 IV push/infusion over 1 to 15 minutes once per day on days 1 & 2
**Less than 10 kg OR younger than 12 months old: 1.25 mg/kg IV push/infusion over 1 to 15 minutes once per day on days 1 & 2
***Administer at a concentration not to exceed 2 mg/mL. Doxorubicin 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) by the following age- and weight-based criteria:
**10 kg or more AND 12 months old or older: 1200 mg/m2 IV over 30 to 60 minutes once on day 1
**Less than 10 kg OR younger than 12 months old: 40 mg/kg IV over 30 to 60 minutes once on day 1
***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 (cycles 1, 3, 5)====
*Mesna (Mesnex) by the following age- and weight-based criteria:
**10 kg or more AND 12 months old or older: 720 mg/m2 IV infusion or IV/PO with cyclophosphamide once on day 1
**Less than 10 kg OR younger than 12 months old: 24 mg/kg IV infusion or IV/PO with cyclophosphamide once on day 1
====Chemotherapy, IE portion (cycles 2, 4, 6)====
*Ifosfamide (Ifex) by the following age- and weight-based criteria:
**10 kg or more AND 12 months old or older: 1800 mg/m2 IV over 60 minutes once per day on days 1 to 5
**Less than 10 kg OR younger than 12 months old: 60 mg/kg IV over 60 minutes once per day on days 1 to 5
***Achieve urine specific gravity less than or equal to 1.010 before starting ifosfamide.
*Etoposide (Vepesid) by the following age- and weight-based criteria:
**10 kg or more AND 12 months old or older: 100 mg/m2 IV over 1 to 2 hours once per day on days 1 to 5
**Less than 10 kg OR younger than 12 months old: 3.3 mg/kg IV over 1 to 2 hours once per day on days 1 to 5
====Supportive therapy, IE portion (cycles 2, 4, 6)====
*Mesna (Mesnex) by the following age- and weight-based criteria:
**10 kg or more AND 12 months old or older: 1080 mg/m2 IV infusion or IV/PO with ifosfamide once per day on days 1 to 5
**Less than 10 kg OR younger than 12 months old: 36 mg/kg IV infusion or IV/PO with ifosfamide once per day on days 1 to 5
14-day cycle for 6 cycles, followed by continuation (to be completed):
===References===
#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 EuroPMC NCT01231906
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Category:Pediatric solid tumors