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小儿局限性尤因肉瘤家族性肿瘤多学科管理中的确定性放疗:结果与预后因素

Definitive irradiation in multidisciplinary management of localized Ewing sarcoma family of tumors in pediatric patients: outcome and prognostic factors.

作者信息

Krasin Matthew J, Rodriguez-Galindo Carlos, Billups Catherine A, Davidoff Andrew M, Neel Michael D, Merchant Thomas E, Kun Larry E

机构信息

Department of Radiological Sciences, Division of Radiation Oncology, St. Jude Children's Research Hospital, 332 North Lauderdale, Memphis, TN 38105-2794, USA.

出版信息

Int J Radiat Oncol Biol Phys. 2004 Nov 1;60(3):830-8. doi: 10.1016/j.ijrobp.2004.04.006.

DOI:10.1016/j.ijrobp.2004.04.006
PMID:15465200
Abstract

PURPOSE

To assess the effect of radiation dose on local tumor control of the Ewing sarcoma family of tumors in 79 patients with localized disease treated at a single institution.

METHODS AND MATERIALS

Thirty-seven patients received vincristine, actinomycin D, cyclophosphamide, and doxorubicin, and 42 received vincristine, actinomycin D, and cyclophosphamide, with alternating cycles of ifosfamide and etoposide; all underwent definitive radiotherapy (median dose, 37.5 Gy) with either low-dose (<40 Gy) or standard dose (> or =40 Gy) radiation delivered according to the protocol. We calculated the cumulative incidence of local treatment failure, disease recurrence, and overall survival and analyzed the effect of known prognostic factors and radiation dose.

RESULTS

The cumulative incidence of local treatment failure at 10 years was 30.4% and that of disease recurrence was 40%. The overall survival rate was 64.5%. Patient age > or =14 years and tumor size > or =8 cm were adverse prognostic factors for local treatment failure; patient age > or =14 years was also associated with worse survival. Although the radiation dose alone did not predict for local treatment failure, the cumulative incidence of local failure at 10 years was 19% when tumors <8 cm were treated with <40 Gy, and no patient treated with standard doses (> or =40 Gy) developed local recurrence (p = 0.084).

CONCLUSION

Tumor size and patient age predict for local tumor control in patients with Ewing sarcoma family of tumors treated with systemic therapy and definitive radiotherapy. Patients treated with reduced-dose radiotherapy experienced unacceptably high rates of local recurrence.

摘要

目的

评估放疗剂量对在单一机构接受治疗的79例局限性尤因肉瘤家族性肿瘤患者局部肿瘤控制的影响。

方法和材料

37例患者接受长春新碱、放线菌素D、环磷酰胺和阿霉素治疗,42例患者接受长春新碱、放线菌素D和环磷酰胺治疗,并交替使用异环磷酰胺和依托泊苷周期;所有患者均按照方案接受了低剂量(<40 Gy)或标准剂量(≥40 Gy)的根治性放疗(中位剂量,37.5 Gy)。我们计算了局部治疗失败、疾病复发和总生存的累积发生率,并分析了已知预后因素和放疗剂量的影响。

结果

10年时局部治疗失败的累积发生率为30.4%,疾病复发的累积发生率为40%。总生存率为64.5%。患者年龄≥14岁和肿瘤大小≥8 cm是局部治疗失败的不良预后因素;患者年龄≥14岁也与较差的生存率相关。虽然单独放疗剂量不能预测局部治疗失败,但当<8 cm的肿瘤接受<40 Gy治疗时,10年时局部失败的累积发生率为19%,而接受标准剂量(≥40 Gy)治疗的患者均未发生局部复发(p = 0.084)。

结论

肿瘤大小和患者年龄可预测接受全身治疗和根治性放疗的尤因肉瘤家族性肿瘤患者的局部肿瘤控制情况。接受低剂量放疗的患者局部复发率高得令人无法接受。

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