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对于低风险滑膜肉瘤的儿童和青少年,单纯手术是足够的治疗方法:来自欧洲儿科软组织肉瘤研究组和儿童肿瘤学组的联合分析。

Surgery alone is sufficient therapy for children and adolescents with low-risk synovial sarcoma: A joint analysis from the European paediatric soft tissue sarcoma Study Group and the Children's Oncology Group.

作者信息

Ferrari Andrea, Chi Yueh-Yun, De Salvo Gian Luca, Orbach Daniel, Brennan Bernadette, Randall R Lor, McCarville M Beth, Black Jennifer O, Alaggio Rita, Hawkins Douglas S, Bisogno Gianni, Spunt Sheri L

机构信息

Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.

Department of Biostatistics, University of Florida, Gainesville, FL, USA.

出版信息

Eur J Cancer. 2017 Jun;78:1-6. doi: 10.1016/j.ejca.2017.03.003. Epub 2017 Apr 7.

Abstract

BACKGROUND

Multimodal risk-adapted treatment is used in paediatric protocols for synovial sarcoma (SS). Retrospective analyses suggest that low-risk SS patients can be safely treated with surgery alone, but no prospective studies have confirmed the safety of this approach. This analysis pooled data from the two prospective clinical trials to assess outcomes in SS patients treated with a surgery-only approach and to identify predictors of treatment failure.

METHODS

Patients with localised SS enrolled on the European paediatric Soft tissue sarcoma Study Group (EpSSG) NRSTS2005 and on the Children Oncology Group (COG) ARST0332 trials, treated with surgery alone were eligible for this analysis. Patients must have undergone initial complete resection with histologically free margins, with a grade 2 tumour of any size or a grade 3 tumour ≤5 cm.

RESULTS

Sixty patients under 21 years of age were eligible for the analysis; 36 enrolled in the COG (from 2007 to 2012) and 24 in the EpSSG study (from 2005 to 2012). The 3-year event-free survival was 90% (median follow-up 5.2 years, range 1.9-9.1). All eight events were local tumour recurrence, whereas no metastatic recurrences were seen. All patients with recurrence were effectively salvaged, resulting in 100% overall survival.

CONCLUSION

This joint prospective analysis showed that patients with adequately resected ≤5 cm SS, regardless of grade, can be safely treated with a surgery-only approach. Avoiding the use of adjuvant chemotherapy and radiotherapy in this low-risk patient population may decrease both short- and long-term morbidity and mortality.

摘要

背景

多模式风险适应性治疗用于小儿滑膜肉瘤(SS)的治疗方案。回顾性分析表明,低风险SS患者单独手术治疗可能安全,但尚无前瞻性研究证实这种方法的安全性。本分析汇总了两项前瞻性临床试验的数据,以评估仅接受手术治疗的SS患者的预后,并确定治疗失败的预测因素。

方法

入选欧洲小儿软组织肉瘤研究组(EpSSG)NRSTS2005和儿童肿瘤学组(COG)ARST0332试验、仅接受手术治疗的局限性SS患者符合本分析条件。患者必须已进行初始完全切除,切缘组织学阴性,肿瘤分级为2级(大小不限)或3级(≤5 cm)。

结果

60例21岁以下患者符合分析条件;36例入选COG试验(2007年至2012年),24例入选EpSSG研究(2005年至2012年)。3年无事件生存率为90%(中位随访5.2年,范围1.9 - 9.1年)。所有8例事件均为局部肿瘤复发,未见远处转移复发。所有复发患者均得到有效挽救,总生存率为100%。

结论

这项联合前瞻性分析表明,≤5 cm的SS患者,无论分级如何,若切除充分,仅接受手术治疗可能安全。在此低风险患者群体中避免使用辅助化疗和放疗可能会降低短期和长期的发病率和死亡率。

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