Introduction: Chest wall Ewing sarcoma (EWS) is a common paediatric malignant tumour affecting the chest wall, accounting for about 10% of all EWS patients. Treatment typically involves intense chemotherapy, complete surgical resection when possible and/or radiation. The EWS of the chest wall has a reported 5-year survival ranging between 40% and 60% in most studies. The following study evaluated the effect of different treatment modalities on survival outcomes in paediatric patients.
Methodology: This is a single-center retrospective cohort study that included 46 patients diagnosed with chest wall EWS between January 2011 and December 2023 at Shaukat Khanum Memorial Cancer Hospital and Research Center. Approval was obtained from the Institutional Review Board for this study. The 5-year follow up period and outcomes were analysed using the SPSS.
Results: The cohort included 46 patients (56% female, 44% male) with a median age of 10 years. Non-metastatic disease was observed in 94% of the patients. The compressed vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide chemotherapy regimen showed better outcomes, with a 48% survival rate compared with 38% for vincristine, doxorubicin, actinomycin, ifosfamide and etoposide. Negative surgical margins were associated with a 56% survival rate. 5-year overall survival (OS) of 42.5% and event-free survival (EFS) of 39%. Both the OS and EFS were better in patients who had surgery alone as local control (LC).
Conclusion: Outcomes in patients who had surgery as LC did better in this study. Early recognition and referral may help in reducing the tumour burden so that good LC with surgical R-zero resection is possible.