International Journal of Hematology and Oncology 2020, Vol 30, Num 1 Page(s): 147-152
Treatment of Extraosseous Ewing Sarcoma in Children: Single Center Experience

Neriman SARI1, Mehmet F. CETINDAG2, Inci E. ILHAN1

1Ankara Oncology Hospital, Department of Pediatric Oncology, Ankara, TURKEY
2Ankara Oncology Hospital, Department of Radiation Oncology, Ankara, TURKEY

Keywords: Extraosseous Ewing sarcoma, Surgery, Chemotherapy, Radiotherapy, Children
Extraosseous Ewing sarcoma (EOES) is an infrequent type of soft tissue sarcoma, which is highly chemosensitive and radiosensitive. It resembles Ewing sarcoma (ES) of bone histopathologically. The purpose of this study was to demonstrate the characteristics and clinical outcomes of children with EOES treated in a single centre, the largest state hospital that accepts oncologic patients. Patients and Method: We analysed data from 13 pediatric patients who were diagnosed with EOES during the period between June 1992 and December 2007. Results: The median age was 12 years (3-16 years) and male/female ratio was 1.2/1. Surgery was planned as a first treatment modality in eight patients in other hospitals. All these patients were admitted to our hospital with local recurrent or progressive disease, except for one with a negative surgical margin. Definitive surgery was performed prior to chemotherapy in four patients and after chemotherapy in three patients. Eleven patients were treated with the EICESS-92 chemotherapy protocol. In 10 patients, radiotherapy was applied to the primary tumor site at a dose of 50-56 Gy. The median follow-up was 48 months (range, 12-103 months). The five-year estimate of failure free survival and overall survival was 67%. None of the patients died due to treatment toxicity. Conclusion: Patients with a diagnosis of EOES must be referred to oncology centers. Treatment must be planned by an oncology team consisting of a pediatric oncologist, an oncological surgeon, and a radiotherapist, in order to avoid mutilating surgery and delays in receiving chemotherapy.