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手足部尤因肉瘤

Ewing sarcoma of the hand or foot.

作者信息

Froeb D, Ranft A, Boelling T, Paulussen M, Klco-Brosius S, Jürgens H, Dirksen U

机构信息

Westfalian Wilhelms University Muenster, Muenster, Germany.

出版信息

Klin Padiatr. 2012 Oct;224(6):348-52. doi: 10.1055/s-0032-1327607. Epub 2012 Nov 9.

Abstract

PURPOSE

Ewing Sarcoma (ES) of the hand or foot is a rare clinical condition. Due to the critical site, it is of major importance to choose an optimal procedure for local control in terms of outcome and function. Local therapeutic options for these patients range from: surgery (OP), surgery followed by radiotherapy (OP & RT), or radiotherapy (RT) alone.

PATIENTS AND METHODS

Data from 80 patients with ES of the hand or foot were analyzed. All patients received chemotherapy according to the protocols of the Cooperative Ewing Sarcoma Study Group (CESS) from 1991 to 2009 (EICESS-92 and EURO-E.W.I.N.G.99). Local therapy consisted of: OP in 39%, OP & RT in 44%, and RT in 12%. In 5% of the patients no local therapy (noL) was performed. Primary endpoint of our study was the event-free-survival (EFS).

RESULTS

The 3-year overall EFS was 62% (95%CI 0.50-0.72). Patients with localized disease had a significantly better outcome with an EFS of 77% (95%CI 0.63-0.86), compared to patients with primary disseminated disease with an EFS of 30% (95%CI 0.14-0.49; p<0.001). In comparing local treatment modalities, no significant difference was observed. The 3-year EFS for OP was 61% (95% CI 0.40-0.76), for OP & RT 66% (95%CI 0.47-0.79) and for RT only 70% (95%CI 0.32-0.89) (p=0.253). Patients who did not receive local treatment had an unfavourable prognosis (3-year EFS=0.25; 95%CI 0.01-0.67; p=0.024). A multivariate analysis which included local treatment modality and known prognosticators, showed that primary dissemination was the only significant prognostic factor.Ewing sarcoma of the hand or foot is associated with a favourable outcome.

CONCLUSION

Our data analysed a limited group of patients and thus did not provide a clear indication for a preferred local treatment modality.

摘要

目的

手足部尤因肉瘤(ES)是一种罕见的临床病症。由于发病部位关键,从治疗效果和功能角度选择最佳的局部控制方法至关重要。这些患者的局部治疗选择包括:手术(OP)、手术后放疗(OP & RT)或单纯放疗(RT)。

患者与方法

分析了80例手足部ES患者的数据。所有患者均根据1991年至2009年合作尤因肉瘤研究组(CESS)的方案(EICESS - 92和EURO - E.W.I.N.G.99)接受化疗。局部治疗方式包括:39%为OP,44%为OP & RT,12%为RT。5%的患者未进行局部治疗(noL)。本研究的主要终点是无事件生存期(EFS)。

结果

3年总EFS为62%(95%CI 0.50 - 0.72)。局限性疾病患者的预后明显更好,EFS为77%(95%CI 0.63 - 0.86),而原发性播散性疾病患者的EFS为30%(95%CI 0.14 - 0.49;p<0.001)。比较局部治疗方式时,未观察到显著差异。OP的3年EFS为61%(95%CI 0.40 - 0.76),OP & RT为66%(95%CI 0.47 - 0.79),单纯RT为70%(95%CI 0.32 - 0.89)(p = 0.253)。未接受局部治疗的患者预后不良(3年EFS = 0.25;95%CI 0.01 - 0.67;p = 0.024)。一项包括局部治疗方式和已知预后因素的多变量分析表明,原发性播散是唯一显著的预后因素。手足部尤因肉瘤的预后良好。

结论

我们的数据分析了有限的一组患者,因此未明确指出首选的局部治疗方式。

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