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[儿童横纹肌肉瘤(RMS)的治疗结果。小儿肿瘤学会软组织肉瘤合作研究(CWS - 81)报告]

[Results of treatment of rhabdomyosarcomas (RMS) in children. A report of the Cooperative Soft Tissue Sarcoma Study (CWS-81) of the Society of Pediatric Oncology].

作者信息

Treuner J, Kaatsch P, Anger Y, Seipp A, Spaar H J, Gerein V, Suder J, Niethammer D

出版信息

Klin Padiatr. 1986 May-Jun;198(3):208-17. doi: 10.1055/s-2008-1026879.

DOI:10.1055/s-2008-1026879
PMID:3523026
Abstract

This analysis refers to 129 children with RMS who were treated between 1981 and 1985 according to the protocol guidelines of the CWS-81 study. The duration of chemotherapy depended on the initial post-operative stage. Patients with stage I and IIA were not to receive any radiotherapy, and patients with primary stage III were treated according to the results of a 16-week chemotherapy treatment: either without radiation (stage Ipc), or with radiotherapy using 40 Gy (stage (IIpc) or 50 Gy (stage IIIpc). The median time of observation was 27 months (Juni 1985). The essential results of the study are as follow: RMS of the extremities in stages I and IIA need radiotherapy, contrary to all other localisations with the same stages. Patients with RMS stage III who are tumor-free after initial chemotherapy (histologically checked) do not need radiotherapy. Patients with microscopic residue (IIpc) or macroscopic residue (IIIpc) after pretreatment showed no difference in their local relapse rate, whereas metastases were found only in the group having macroscopic tumor residue up to week 16. An additional examination of this study observed tumor response during initial chemotherapy and its relationship to prognosis. This analysis showed that the degree of tumor regression per unit of time permits the most favorable prognostic statement. Patients with clinical complete remission after 7/9 weeks showed a 100% relapse-free chance of survival, independent of localisation, tumor size or histological subtype. Those with a tumor reduction of greater than 2/3 but no complete remission showed 67% chance of survival, and those with greater than 1/3-2/3 tumor reduction had 25%. Tumor response kinetics under initial chemotherapy allows better individual therapy in the future. The overall result of the study concerning localisation and stage corresponds to that of the IRS I and II studies. Patients with undifferentiated sarcomas, extraossary Ewing's sarcoma and synovial sarcomas can be treated according to the same principles as RMS, since no significant differences in prognosis could be found.

摘要

该分析涉及129例横纹肌肉瘤患儿,他们于1981年至1985年间按照CWS - 81研究的方案指南接受治疗。化疗疗程取决于初始术后分期。I期和IIA期患者不接受任何放疗,原发性III期患者根据16周化疗治疗结果进行治疗:要么不进行放疗(Ipc期),要么采用40 Gy放疗(IIpc期)或50 Gy放疗(IIIpc期)。观察的中位时间为27个月(1985年6月)。该研究的主要结果如下:与相同分期的所有其他部位相反,I期和IIA期肢体横纹肌肉瘤需要放疗。初始化疗后(经组织学检查)无肿瘤的III期横纹肌肉瘤患者不需要放疗。预处理后有镜下残留(IIpc)或肉眼残留(IIIpc)的患者,其局部复发率无差异,而仅在直至第16周有肉眼肿瘤残留的组中发现转移。该研究的另一项检查观察了初始化疗期间的肿瘤反应及其与预后的关系。该分析表明,单位时间内肿瘤消退程度可做出最有利的预后判断。7/9周后临床完全缓解的患者,无论肿瘤部位、大小或组织学亚型如何,其无复发生存几率为100%。肿瘤缩小大于2/3但未完全缓解的患者,生存几率为67%,肿瘤缩小大于1/3 - 2/3的患者,生存几率为25%。初始化疗期间的肿瘤反应动力学有助于未来实现更好的个体化治疗。该研究关于肿瘤部位和分期的总体结果与IRS I和II研究的结果一致。未分化肉瘤、骨外尤文肉瘤和滑膜肉瘤患者可按照与横纹肌肉瘤相同的原则进行治疗,因为未发现预后有显著差异。

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[Results of treatment of rhabdomyosarcomas (RMS) in children. A report of the Cooperative Soft Tissue Sarcoma Study (CWS-81) of the Society of Pediatric Oncology].[儿童横纹肌肉瘤(RMS)的治疗结果。小儿肿瘤学会软组织肉瘤合作研究(CWS - 81)报告]
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