Multimodality treatment of pediatric Ewing sarcoma: A single-center 10-year analysis of outcomes

Published:August 04, 2022DOI:



      Ewing sarcoma, a malignancy originating from the bone or soft tissues most commonly diagnosed in adolescents, requires multimodality therapy. Although both surgical resection and radiation therapy are effective local control modalities, there are limited data comparing outcomes in patients treated with surgery versus radiation. We sought to determine whether there were differences in 5-year local failure-free survival, event-free survival, and overall survival based on the modality used for local control.


      Patients treated for Ewing sarcoma at a single tertiary pediatric hospital between 2010 and 2020 were included for retrospective analysis. Patient and tumor demographics, treatment information, and patient response to therapies were collected from the medical record. Outcome measures were local failure-free survival, event-free survival, and overall survival at 5 years from diagnosis.


      Sixty-one patients met inclusion criteria. All patients received chemotherapy, and 68.9% of patients presented with localized disease. Of these, 23.8% were treated with radiation alone; the remaining 76.2% underwent resection ± radiation. A total of 52.4% of patients with localized disease achieved R0 resection. Only 3 patients experienced local progression; there was no difference between treatment groups. There was no significant association between local control modality and event-free survival or overall survival in patients with localized disease, regardless of margin status.


      There was no significant difference in 5-year local failure-free survival, event-free survival, or overall survival in Ewing sarcoma patients treated with radiation versus surgery ± radiation, regardless of whether or not R0 resection was achieved. Future directions include a multi-institutional study to allow for further subgroup analysis and increased sample size.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Smith M.A.
        • Altekruse S.F.
        • Adamson P.C.
        • Reaman G.H.
        • Seibel N.L.
        Declining childhood and adolescent cancer mortality.
        Cancer. 2014; 120: 2497-2506
        • American Cancer Society
        Survival Rates for Ewing Tumors.
        (Updated May 25, 2021)
        • Thorpe S.W.
        • Weiss K.R.
        • Goodman M.A.
        • Heyl A.E.
        • McGough R.L.
        Should aggressive surgical local control be attempted in all patients with metastatic or pelvic Ewing’s sarcoma?.
        Sarcoma. 2012; 2012953602
        • Miller B.J.
        • Gao Y.
        • Duchman K.R.
        Does surgery or radiation provide the best overall survival in Ewing's sarcoma? A review of the National Cancer Data Base.
        J Surg Oncol. 2017; 116: 384-390
        • Yock T.I.
        • Krailo M.
        • Fryer C.J.
        • et al.
        Local control in pelvic Ewing sarcoma: analysis from INT-0091—a report from the Children’s Oncology Group.
        J Clin Oncol. 2006; 24: 3838-3843
        • Werier J.
        • Yao X.
        • Caudrelier J.M.
        • et al.
        A systematic review of optimal treatment strategies for localized Ewing’s sarcoma of bone after neo-adjuvant chemotherapy.
        Surg Oncol. 2016; 25: 16-23
        • Hesla A.C.
        • Papakonstantinou A.
        • Tsagkozis P.
        Current status of management and outcome for patients with Ewing sarcoma.
        Cancers (Basel). 2021; 13
      1. American Cancer Society. Radiation Therapy Side Effects. Updated December 10, 2020. Accessed February 18, 2022.

        • Ozaki T.
        • Hillmann A.
        • Hoffmann C.
        • et al.
        Ewing’s sarcoma of the femur: prognosis in 69 patients treated by the CESS group.
        Acta Orthop Scand. 1997; 68: 20-24
        • DuBois S.G.
        • Krailo M.D.
        • Gebhardt M.C.
        • et al.
        Comparative evaluation of local control strategies in localized Ewing sarcoma of bone: a report from the Children’s Oncology Group.
        Cancer. 2015; 121: 467-475
        • Bacci G.
        • Ferrari S.
        • Longhi A.
        • et al.
        Local and systemic control in Ewing’s sarcoma of the femur treated with chemotherapy, and locally by radiotherapy and/or surgery.
        J Bone Joint Surg Br. 2003; 85: 107-114
        • Bacci G.
        • Longhi A.
        • Briccoli A.
        • Bertoni F.
        • Versari M.
        • Picci P.
        The role of surgical margins in treatment of Ewing’s sarcoma family tumors: experience of a single institution with 512 patients treated with adjuvant and neoadjuvant chemotherapy.
        Int J Radiat Oncol Biol Phys. 2006; 65: 766-772
        • Carrie C.
        • Mascard E.
        • Gomez F.
        • et al.
        Nonmetastatic pelvic Ewing sarcoma: report of the French society of pediatric oncology.
        Med Pediatr Oncol. 1999; 33: 444-449
        • Shankar A.G.
        • Pinkerton C.R.
        • Atra A.
        • et al.
        Local therapy and other factors influencing site of relapse in patients with localised Ewing’s sarcoma. United Kingdom Children’s Cancer Study Group (UKCCSG).
        Eur J Cancer. 1999; 35: 1698-1704
        • Ozaki T.
        • Hillmann A.
        • Hoffmann C.
        • et al.
        Significance of surgical margin on the prognosis of patients with Ewing’s sarcoma: a report from the Cooperative Ewing’s Sarcoma Study.
        Cancer. 1996; 78: 892-900
        • Lin P.P.
        • Jaffe N.
        • Herzog C.E.
        • et al.
        Chemotherapy response is an important predictor of local recurrence in Ewing sarcoma.
        Cancer. 2007; 109: 603-611
      2. Children’s Oncology Group. AEWS1031: A Phase III Randomized Trial of Adding Vincristine-topotecan-cyclophosphamide to Standard Chemotherapy in Initial Treament of Non-Metastatic Ewing Sarcoma. Accessed February 21, 2022.

        • Children’s Oncology Group
        AEWS1221: Randomized Phase 3 Trial Evaluating the Addition of IFG-1R Monoclonal Antibody Ganitumab to Multi-Agent Chemotherapy for Patients with Newly Diagnosed Metastatic Ewing Sarcoma.
        Date: 2014
        Date accessed: April 21, 2022
        (Updated October 30, 2018)
        • Craft A.
        • Cotterill S.
        • Malcolm A.
        • et al.
        Ifosfamide-containing chemotherapy in Ewing’s sarcoma: the Second United Kingdom Children’s Cancer Study Group and the Medical Research Council Ewing’s Tumor Study.
        J Clin Oncol. 1998; 16: 3628-3633
        • Womer R.B.
        • West D.C.
        • Krailo M.D.
        • et al.
        Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children’s Oncology Group.
        J Clin Oncol. 2012; 30: 4148-4154
        • Arai Y.
        • Kun L.E.
        • Brooks M.T.
        • et al.
        Ewing’s sarcoma: local tumor control and patterns of failure following limited-volume radiation therapy.
        Int J Radiat Oncol Biol Phys. 1991; 21: 1501-1508
        • Rodriguez-Galindo C.
        • Navid F.
        • Liu T.
        • Billups C.A.
        • Rao B.N.
        • Krasin M.J.
        Prognostic factors for local and distant control in Ewing sarcoma family of tumors.
        Ann Oncol. 2008; 19: 814-820
        • Tepper J.E.
        • Suit H.D.
        Radiation therapy alone for sarcoma of soft tissue.
        Cancer. 1985; 56: 475-479
        • Spalek M.J.
        • Kozak K.
        • Czarnecka A.M.
        • Bartnik E.
        • Borkowska A.
        • Rutkowski P.
        Neoadjuvant treatment options in soft tissue sarcomas.
        Cancers (Basel). 2020; 12