International Journal of Hematology and Oncology 2020, Vol 30, Num 2 Page(s): 178-187
EVALUATION OF EWING SARCOMA FAMILY TUMORS IN CHILDREN

REJİN KEBUDİ1, ESRA KAYTAN1, İNCİ AYAN1, FERHAN AKINCİ1, GÜLNUR TOKUÇ1, ÖMER GÖRGÜN1, HARZEM ÖZGER1, BİLGE BİLGİÇ1, VAKUR OLGAÇ1, EMİN DARENDELİLER1

İstanbul Üniversitesi Onkoloji Enstitüsü, Pediatrik Onkoloji Bilim Dalı

Keywords: ewing sarcoma, prognostic factors, treatment
The aim of this study is to review Ewing sarcoma family tumors (ESFT) in regard to demographic features, response to initial chemotherapy, survival and prognostic factors. Seventy-three children (F;M=36;37) with a median age of 12 years (3-16) were admitted between September 1989 and May 2000. There were 55 localized and 18 metastatic patients. Primary localization was in the extremities in 30 and other sites in 43 patients. Fifty one patients were osseous and 22 extraosseous. Initial treatment consisted of 3 cycles of IE/VAC (ifosfamide, etoposide, vincristine, adriamycine/actinomycin D, cyclophosphamide) chemotherapy; followed by local therapy [radiotherapy (RT) ± surgery (S)] at week 9; and adjuvant chemotherapy for a total of 1 year. The objective clinical and radiological response rate to three courses of initial chemotherapy was 80.5%. RT was performed for local control in 34, S in 8, RT + S in 21 patients. Nineteen patients had progressive disease or relapse (6 local, 12 distant, 1 local + distant) at a median time of 17 (4-41) months. The overall survival for the whole group at 2 years and 5 years is 65% and 51% respectively. Metastasis at diagnosis (5 years survival 22% vs 63%, p=0.02) and poor response to initial therapy (5 years survival 54% vs 2 years survival 20%, p=0.01) were found to be poor prognostic factors. Age, gender, localisation, osseous/extraosseous tumor and tumor size were not found to be prognostic factors. In conclusion, in this series, survival of children with ESFT was found to be comparable to other studies. Metastasis at diagnosis and response to initial treatment were the most important prognostic factors.