Combined Modality Therapy of Ewing Sarcoma / 대한소아혈액종양학회지

ABSTRACT

PURPOSE: Ewing sarcoma (ES) occurs mainly in the pediatric and adolescent population. We evaluated the clinical characteristics and the efficacy of treatment on ES in children who were treated at Seoul National University Children's Hospital. METHODS: We undertook a retrospective analysis of 12 patients diagnosed as ES between March, 1986 and December, 2002. Systemic chemotherapyregimens were as follows CCG 731 (Vincristine, Adriamycin, Methotrexate, Actinomycin D, Cyclophosphamide; N=6), CCG 7881B (Ifosfamide, Etoposide, Vincristine, Adriamycin, Cyclophosphamide; N=2), POG-ICE (Ifosfamide, Etoposide, Carboplatin; N=1), CCG 7881B and BCD (Bleomycin, Cyclophosphamide, Actinomycin D; N=1), CCG 7881B, POG-ICE and VEC (Vincristine, Etoposide, Carboplatin; N=1). One case received CCG 7881B and autologous peripheral blood stem cell transplantation (PBSCT). RESULTS: Among the 12 patients, 8 patients were male and the others were female. The patients median age was 7 years 10 months. The primary tumors occurred in femur (N=4), pubis (N=2), vertebra (N=2) and clavicle, humerus, rib, phalanx one each. All patients were treated with systemic chemotherapy and the duration was median 13 months. Eight patients received radical surgery and nine patients received radiotherapy. Overall 5 years survival rate was 33.3% and one patient who received systemic chemotherapy and PBSCT is alive disease-free for more than 5 years. Eight patients who had more than one poor prognostic factor died. CONCLUSION: Though recent therapeutic trials led to improvement of survival in patients with ES, the prognosis for patient with high-risk disease remain poor and the treatment related toxicities are problem to solve. For the advanced or recurrent cases more intensive therapeutic modality such as autologous bone mallow transplantation or PBSCT should be considered.