Dose-Intensified Compared With Standard Chemotherapy for Nonmetastatic Ewing Sarcoma Family of Tumors: A Children's Oncology Group Study
Dose-Intensified Compared With Standard Chemotherapy for Nonmetastatic Ewing Sarcoma Family of Tumors: A Children's Oncology Group Study
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DOI：
10.1200/jco.2008.19.1478
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发表时间：
2009-05-20
影响因子：
45.3
通讯作者：
Grier, Holcombe E.
中科院分区：
文献类型：
--
作者：
Granowetter, Linda;Womer, Richard;Grier, Holcombe E.
尤文肉瘤家族肿瘤（Ewing sarcoma family of tumors，ESFT）是一组发生于软组织和骨的恶性肿瘤，具有EWS位点的染色体易位。组间研究INT-0091证明了长春新碱、环磷酰胺、多柔比星（VDC）和更生霉素交替异环磷酰胺和依托泊苷（IE）治疗方案在治疗非转移性骨ESFT患者方面优于VDC。本研究的目的是确定是否与IE交替VDC的剂量强化方案将进一步改善非转移性ESFT的骨或软组织的患者的结果。他们被随机分配到接受标准剂量的VDC/IE超过48周或剂量强化方案的VDC/IE超过30 weeks.ResultsFour 78例患者符合资格要求：231例患者接受了标准方案; 247例患者接受了强化方案。所有合格患者的5年无事件生存率（EFS）和总生存率分别为71.1%（95% CI，67.7%至75.0%）和78.6%（95% CI，74.6%至82.1%）。接受标准方案（5年EFS，72.1%; 95% CI，65.8%-77.5%）或强化方案（5年EFS，70.1%; 63.9%-75%）治疗的患者之间的EFS无显著差异（P = 0.57）。软组织肿瘤患者占研究人群的20%，有软组织和骨原发sites.Conclusiondose的烷化剂在本试验中测试的患者之间的结果没有差异，并没有改善非转移性ESFT的骨或软组织的患者的结果。
PurposeThe Ewing sarcoma family of tumors (ESFT) is a group of malignant tumors of soft tissue and bone sharing a chromosomal translocation affecting the EWS locus. The Intergroup INT-0091 demonstrated the superiority of a regimen of vincristine, cyclophosphamide, doxorubicin (VDC), and dactinomycin alternating with ifosfamide and etoposide (IE) over VDC for patients with nonmetastatic ESFT of bone. The goal of this study was to determine whether a dose-intensified regimen of VDC alternating with IE would further improve the outcome for patients with nonmetastatic ESFT of bone or soft tissue.MethodsPatients with previously untreated, nonmetastatic ESFT of bone or soft tissue were eligible. They were randomly assigned to receive standard doses of VDC/IE over 48 weeks or a dose-intensified regimen of VDC/IE over 30 weeks.ResultsFour hundred seventy-eight patients met eligibility requirements: 231 patients received the standard regimen; 247 patients received the intensified regimen. The 5-year event-free survival (EFS) and overall survival rates for all eligible patients were 71.1% (95% CI, 67.7% to 75.0%) and 78.6% (95% CI, 74.6% to 82.1%), respectively. There was no significant difference (P = .57) in EFS between patients treated with the standard (5-year EFS, 72.1%; 95% CI, 65.8% to 77.5%) or intensified regimen (5-year EFS, 70.1%; 63.9% to 75%). Patients with soft tissue tumors accounted for 20% of the study population; there was no difference in outcome between patients with soft tissue and bone primary sites.ConclusionDose escalation of alkylating agents as tested in this trial did not improve the outcome for patients with nonmetastatic ESFT of bone or soft tissue.