Outcome of Ewing sarcoma in children: Twenty years experience from a single center

Year 2024, , 373 - 378, 30.10.2024

Nurşah Eker , Gizem Tanali Ömer Sofulu , Zerrin Ozgen Kemal Turkoz Ayse Gulnur Tokuc

https://doi.org/10.5472/marumj.1573692

Abstract

ABSTRACT
Objective: Ewing sarcoma (ES) is a significant malignancy in pediatric patients, with a notable impact on bone health. Despite advances
in treatment, ES still poses challenges, particularly in cases of metastasis or relapse. This study aims to evaluate the outcomes of ES in
children treated at our center over a twenty-year period.
Patients and Methods: We retrospectively reviewed pediatric patients diagnosed with ES at our center between January 2004 and
February 2024. Data including demographic information, tumor characteristics, treatment modalities, and survival outcomes were
analyzed.
Results: Among 986 pediatric solid tumor cases, 137 (13.8%) were diagnosed with ES. After excluding ineligible cases, 115 ES cases
were included in the study. The most common sites of involvement were the lower extremities. Metastatic disease was observed
in 35.8% of cases, with the lungs being the most common site. Advanced age, and pelvic involvement were associated with poor
prognosis. Histopathological response to neoadjuvant chemotherapy, represented by tumor necrosis rate, metastatic and relapse
disease significantly influenced survival outcomes.
Conclusion: Despite multimodal therapies, ES in children, especially with metastatic disease or relapse, presents a challenging
prognosis. Early diagnosis and the development of novel treatment strategies are imperative to improve outcomes for these patients.

Keywords

Ewing sarcoma, Children, Survival, Prognosis

References

• Davis L, Malempati S. Ewing sarcoma in adolescents and young adults: diagnosis and treatment. Clinical Oncology in Adolescents and Young Adults 2014; 4: 21-31. doi: 10.2147/ COAYA.S61451.
• Umeda K, Miyamura T, Yamada K, et al. Clinical outcome of patients with recurrent or refractory localized Ewing’s sarcoma family of tumors: A retrospective report from the Japan Ewing Sarcoma Study Group. Cancer Rep 2021;4: 1329. doi: 10.1002/cnr2.1329.
• Collier AB, Krailo MD, Dang HM, et al. Outcome of patients with relapsed or progressive Ewing sarcoma enrolled on cooperative group phase 2 clinical trials: A report from the Children’s Oncology Group. Pediatr Blood Cancer 2021; 68:4- 8. doi: 10.1002/pbc.29333.
• Werier J, Yao X, Caudrelier JM, et al. A systematic review of optimal treatment strategies for localized Ewing’s sarcoma of bone after neo-adjuvantchemotherapy. Surg Oncol 2016; 25: 16-23. doi: 10.1016/j.suronc.2015.11.002
• Riggi N, Suvà ML, Stamenkovic I. Ewing’s Sarcoma. New England Journal of Medicine 2021; 384:154-64. doi: 10.1056/ NEJMra2028910.
• Bosma SE, Ayu O, Fiocco M, Gelderblom H, Dijkstra PDS. Prognostic factors for survival in Ewing sarcoma: A systematic review. Surg Oncol 2018; 27: 603-10. doi. 10.1016/j. suronc.2018.07.016.
• Lynch AD, Gani F, Meyer cF, Morris cD, Ahuja N, Johnston FM. Extraskeletal versus Skeletal Ewing Sarcoma in the adult population: controversies in care. Surg Oncol 2018;27: 373-9. doi: 10.1016/j.suronc.2018.05.016.
• Paulussen M, Ahrens S, Craft AW, et al. Ewing’s tumors with primary lung metastases: survival analysis of 114 (European Intergroup) cooperative Ewing’s Sarcoma Studies patients. J Clin Oncol 1998; 16:3044. doi: 10.1200/JCO.1998.16.9.3044.
• Jakutis G, Ragelienė L, Rascon J. Survival of children treated for Ewing sarcoma in Lithuania: a single center experience. Acta Medica Litu 2018; 24: 199-208. doi: 10.6001/actamedica. v24i4.3615.
• Balamuth NJ, Womer RB. Ewing’s sarcoma. Lancet Oncol 2010; 11:184-9. doi: 10.1016/S1470-2045(09)70286-4.
• Stahl M, Ranft A, Paulussen M, et al. Risk of recurrence and survival after relapse in patients with Ewing sarcoma. Pediatr Blood Cancer 2011; 57:549-53. doi: 10.1002/pbc.23040.
• Bacci G, Ferrari S, Longhi A, et al. Therapy and survival after recurrence of Ewing’s tumors: The Rizzoli experience in 195 patients treated with adjuvant and neoadjuvant chemotherapy from 1979 to 1997. Ann Oncol 2003; 14:1654-99. doi: 10.1093/ annonc/mdg457.
• Leavey PJ, Mascarenhas L, Marina N, et al. Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi-modality therapy: A report from the children’s oncology group. Pediatr Blood Cancer 2008; 51:334-8. doi: 10.1002/pbc.21618.
• Granowetter L, Womer R, Devidas M, et al. Dose-intensified compared with standard chemotherapy for nonmetastatic Ewing sarcoma family of tumors: a Children’s Oncology Group Study. J Clin Oncol 2009; 27:2536-41.
• Rodrı´guez-Galindo C, Liu T, Krasin MJ, et al. Analysis of prognostic factors in Ewing sarcoma family of tumours: Review of St. Jude Children’s Research Hospital studies. Cancer 2007; 110: 375-84. doi: 10.1002/cncr.22821.
• Jenkin RD, Al-Fawaz I, Al-Shabanah M, et al. Localised Ewing sarcoma/PNET of bone: Prognostic factors and international data comparison. Med Pediatr Oncol 2002; 39: 586-93. doi: 10.1002/mpo.10212.
• Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumour of bone. N Engl J Med 2003; 348: 694-701. doi: 10.1056/NEJMoa020890.
• Cash T, McIlvaine E, Krailo MD, et al. Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children’s Oncology Group. Pediatr Blood Cancer 2016;63: 1771–9. doi: 10.1002/pbc.26096.
• Ghandour M, Lehner B, Klotz M, et al. Extraosseous Ewing sarcoma in children: A systematic review and meta-analysis of clinicodemographic characteristics. Children 2022; 9: 1859. doi: 10.3390/children9121859.
• Tokuç G, Yılmaz B. Ewing sarcoma. Çocuklarda Kemik Tümörleri. 1. Baskı. Ankara: Türkiye Klinikleri, 2021:36-44.
• Majeed SS, Hawzheen AM, Ali JS, et al. Treatment Outcomes of Pediatric Patients With Ewing Sarcoma in a War-Torn Nation: A Single-Institute Experience From Iraq. J Glob Oncol 2019; 5: 1-9. doi: 10.1200/JGO.18.00122.
• Al Odaman I, Demirağ B, Sabah D, et al. Clinical Features, Prognostic Factors and Outcome of Children with Ewing Sarcoma: A Single-center Experience. J Curr Pediatr 2023; 21: 60-8. doi: 10.4274/jcp.2023.46667.
• Lozano-Calderon SA, Albergo JI, Groot OQ, et al. Complete tumor necrosis after neoadjuvant chemotherapy defines good responders in patients with Ewing sarcoma. Cancer 2023; 129:60-70. doi: 10.1002/cncr.34506.
• Oberlin O, Deley ML, Bui BN, et al. Prognostic factors in localized Ewing’s tumors and peripheral neuroectodermal tumors: the third study of the French Society of Pediatric Oncology (EW88 study). Br J Cancer 2001; 85:1646-54. doi: 10.1054/bjoc.2001.2150.