Combined Modality Therapy of Ewing Sarcoma / 대한소아혈액종양학회지

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.

Reliability of Diastolic Flow Velocity of the Left Pulmonary Artery for the Diagnosis of Patent Ductus Arteriosus in Preterm Infants

PURPOSE: Diagnosis of a hemodynamically significant patent ductus arteriosus (PDA) that should be treated is difficult to determine by clinical and echocardiographic examination. The purpose of this study is to clarify the usefulness of diastolic flow velocity (DFV) of the left pulmonary artery (LPA) determined by echocardiography in the assessment of significant PDA in preterm infants. METHODS: Clinical and echocardiographic findings, including DFV in LPA, of PDA were evaluated at 24 hours, 48-72 hours of age and after indomethacin treatment in thirty-nine infants ranging from 25 to 34 gestational weeks of age. DFVs of the study group (N=13) with significant PDA were compared with those of healthy control group (N=26) without significant PDA. RESULTS: DFVs in healthy preterm infants were high in the first few days and were significantly decreased after spontaneous ductal closure. DFVs in preterm infants with significant PDA who underwent indomethacin treatment were significantly higher than that of healthy control infants. After indomethacin treatment, DFVs in this study group remained high with continuing significant PDA and markedly decreased with disappearance of significant PDA. Until ductus arteriosus closed, DFVs showed a significant correlation with the magnitudes of ductal shunt. A cutoff value for DFV of 30 cm/sec showed a sensitivity of 77% and a specificity of 92% as a predictor of significnat PDA in preterm infants. CONCLUSION: Measurement of DFV in LPA by echocardiography is a useful method for assessing the significnat PDA which may require treatment in preterm infants.