Real world data of Ewing sarcoma from a resource-limited setting with poor compliance to treatment leading to poor outcomes

Background: There is limited data from India on Ewing sarcoma (ES) patients. We analysed the demographic and clinical profile of ES patients, the systemic chemotherapy, local treatment and outcomes in patients with localised, metastatic and recurrent disease. Methods: Data of ES patients reporting from 2010 to 2019 to a tertiary care referral centre in north India was evaluated. A total of 81 patients were retrieved of whom 76 were assessed for treatment and outcomes. Patients were stratified as per localised (LD) or metastatic disease (MD). Outcomes were evaluated in terms of 3-year and 5-year disease-free survival (DFS) and overall survival (OS). Prognostic factors influencing OS for patients with LD were assessed. Results: The majority (68%, n = 55) of patients presented from rural areas with 30% (n = 24) presenting 6 months after the onset of symptoms, 63% (n = 51) had primary tumours more than 8 cm and 7% (n = 6) had a pathological fracture at presentation, 78% (n = 63) patients had LD while 22% (n = 18) patients had MD. Local treatment consisted of surgery in 56% (n = 28) patients and definitive radiotherapy in 44% (n = 22) patients. Compliance with chemotherapy was poor with patients receiving a median of five and seven cycles of chemotherapy as neoadjuvant and consolidation chemotherapy, respectively. Three-year OS for LD, MD and overall cohort was 41%, 6% and 32%, respectively. Size of the primary tumour > 8 cm, completion of less than 15 cycles of chemotherapy and presence of MD was associated with inferior survival on multivariate analysis. Conclusion: Poor outcomes were reported in this cohort of ES patients from a resource- limited setting where patients have a delayed diagnosis, advanced disease and poor compliance to adjuvant consolidation chemotherapy secondary to geographical, social and financial barriers. There is an urgent need to address these barriers for low middle- income countries to improve outcomes.