Rare Presentations and the Management of Cases of Ewing's Sarcoma - In a Tertiary Care Centre of Mangalore

To present a comprehensive overview of the clinical presentations, diagnostic approaches, treatment modalities, and outcomes in a series of cases of Ewing's sarcoma in the pediatric and adolescent age group.

We identified all patients with primary Ewing's sarcoma who were treated at our teaching hospital from 2017 to 2024 with an average follow-up of 2 years. A structured data collection form was used to record available information on demographic variables, clinical presentation, investigations (X-ray, computed tomography scans, magnetic resonance imaging scans, and positron emission tomography scans), laboratory findings, histopathology, diagnosis, and any associated clinical findings. In addition, the outcome of various treatment modalities on Ewing's sarcoma evaluated by the response evaluation criteria in solid tumors (RECIST 1.1) was also recorded.

The most common anatomical site was the ribs and intercostal space, followed by the left ethmoid sinus. The peak incidence was between 11 and 15 years of age, and the most common age of onset was <15 years of age. Immunohistochemical analysis was done in 66.7% of patients. All patients underwent chemotherapy, and 66.7% developed complications. The tumor increased in size by 20% in 41.7% of patients, and a reduction of 30% was also observed in 41.7% of patients. The outcome, as evaluated by RECIST, was not associated with any variable.

It can be concluded that Ewing's sarcoma is an aggressive tumor that needs to be diagnosed early and managed using a multidisciplinary approach. Given the rare nature of the disease, there are no agreed-upon guidelines for effective management. There is an urgent need to establish a new regimen that includes molecule-based pathological diagnosis and standardized treatment protocol.