Raciborska Anna, Bilska Katarzyna, Drabko Katarzyna, Rogowska Elżbieta, Chaber Radosław, Pogorzała Monika, Wyrobek Elżbieta, Połczyńska Katarzyna, Rodriguez-Galindo Carlos, Woźniak Wojciech
Klinika Chirurgii Onkologicznej Dzieci i Młodzieży, Instytut Matki i Dziecka w Warszawie, Warszawa.
Med Wieku Rozwoj. 2013 Apr-Jun;17(2):117-25.
Patients with metastatic, progressive or recurrent Ewing sarcoma have a poor prognosis. In addition to increasing the intensity of conventional chemotherapy, the combination of irinotecan and temozolomide has been proposed as an effective salvage regimen for some pediatric malignancies.
To evaluate the effect of two different salvage regimens on the final outcome of patients with refractory Ewing sarcoma.
During the period 2008-2012, twenty-two patients (age between 2.9 -19.3 years) with recurrent or refractory Ewing sarcoma were treated with the combination of vincristine, irinotecan and temozolomide (VIT regimen), and twenty patients were treated with the combination of cisplatin, doxorubicin, cyclophosphamide and teniposide (PACE regimen). All patients had standard tumour imaging and laboratory evaluation. All toxicities were documented. The WHO criteria were used to evaluate response. Statistical analysis was performed using STATA 10.0 for Windows. Results distributions were estimated using the method of Kaplan-Meier. The log-rang test was used to compare the groups.
A total of 91 cycles of VIT and 65 cycles of PACE were administered. For VIT therapy the overall response rate was 68.1%. Median time to progression was 3.0 months. Five patients are alive with no evidence of disease with a median follow-up of 10.3 months. For PACE therapy the overall response rate was 75%. Median time to progression was 3.5 months. Four patients are alive with no symptoms of disease with a median follow-up of 17.6 months. The 2 years overall survival probability after recurrence was 29.94%; no differences were detected between therapy groups. Toxicity for PACE was significantly higher.
The effectiveness of VIT regimen in refractory Ewing Sarcoma is comparable to conventional chemotherapy. The VIT regimen has less associated toxicities than the PACE regimen.
转移性、进展性或复发性尤因肉瘤患者预后较差。除了增加传统化疗的强度外，伊立替康和替莫唑胺联合用药已被提议作为一些儿童恶性肿瘤的有效挽救方案。
评估两种不同挽救方案对难治性尤因肉瘤患者最终结局的影响。
在2008年至2012年期间，22例（年龄在2.9至19.3岁之间）复发性或难治性尤因肉瘤患者接受了长春新碱、伊立替康和替莫唑胺联合治疗（VIT方案），20例患者接受了顺铂、多柔比星、环磷酰胺和替尼泊苷联合治疗（PACE方案）。所有患者均进行了标准的肿瘤影像学和实验室评估。记录所有毒性反应。采用WHO标准评估疗效。使用Windows版STATA 10.0进行统计分析。采用Kaplan-Meier方法估计结果分布。使用对数秩检验比较各组。
共给予91个周期的VIT方案和65个周期的PACE方案。VIT治疗的总缓解率为68.1%。中位进展时间为3.0个月。5例患者存活，无疾病证据，中位随访时间为10.3个月。PACE治疗的总缓解率为75%。中位进展时间为3.5个月。4例患者存活，无疾病症状，中位随访时间为17.6个月。复发后2年总生存概率为29.94%；治疗组之间未检测到差异。PACE方案的毒性明显更高。
VIT方案在难治性尤因肉瘤中的有效性与传统化疗相当。VIT方案的相关毒性低于PACE方案。
