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[CWS 81研究范围内非横纹肌肉瘤性软组织肿瘤的治疗结果]

[Results of the treatment of non-rhabdomyosarcomatous soft tissue neoplasms within the scope of the CWS 81 study].

作者信息

Treuner J, Suder J, Gerein V, Beck J, Winkler K, Weinel F, Gnekow A, Niethammer D

出版信息

Klin Padiatr. 1987 May-Jun;199(3):209-17. doi: 10.1055/s-2008-1026792.

Abstract

347 children with all types of soft tissue sarcoma (STS) have been entered between 1981 and April 1986 into the CWS-81 study. Excluding 229 patients with rhabdomyosarcoma, 118 patients with other STS were analysed in the histological subgroups, separately. 33 synovial sarcomas (SyS), 15 undifferentiated sarcomas (US) and 13 extrasceletal Ewing's sarcoma (EES) were treated with a multimodale trial including chemotherapy and radiotherapy after initial not-mutilating resection or biopsy only, Relapse-free survival rate by Kaplan-Meier was 66% in SyS, 48% in EES and 38% in US, respectively. Patients treated according to the guidelines of the CWS-protocol showed better prognosis than patients with major protocol violations or patients with recurrence when entered into the trial. In SyS only 2 of the 7 patients with recurrence developed metastases. The tumor response rate within 7-9 weeks chemotherapy exclusively given was 71% in patients with stage III and IV. Non-responding tumors to chemotherapy had bad prognosis, irrespective of histological subtype or further treatment. EES and US developed earlier recurrence and more often metastases than SyS or rhabdomyosarcomas. The administered VACA-chemotherapy seems not to be very effective in EES and US. In all other varieties of STS recurrence at the primary site assumed relatively greater importance. Chemotherapy and radiation were not administered in all patients. Fibrosarcoma (FS) was diagnosed in 13 patients. Six of these were entered in the trial at the time of local recurrence and 4 of these 6 relapsed again locally, one with metastasis concurrently.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

1981年至1986年4月期间,347例各种类型软组织肉瘤(STS)患儿纳入了CWS - 81研究。排除229例横纹肌肉瘤患者后,对118例其他类型STS患者按组织学亚组分别进行分析。33例滑膜肉瘤(SyS)、15例未分化肉瘤(US)和13例骨外尤文肉瘤(EES)接受了多模式试验治疗,初始仅进行非致残性切除或活检后,再进行化疗和放疗。采用Kaplan - Meier法计算的无复发生存率,SyS为66%,EES为48%,US为38%。按照CWS方案指南治疗的患者预后优于试验入组时严重违反方案或复发的患者。在SyS中,7例复发患者仅2例发生转移。仅接受7 - 9周化疗的III期和IV期患者肿瘤缓解率为71%。化疗无反应的肿瘤预后不良,与组织学亚型或进一步治疗无关。EES和US比SyS或横纹肌肉瘤更早复发且更常发生转移。所给予的VACA化疗对EES和US似乎效果不佳。在所有其他类型的STS中,原发部位复发相对更为重要。并非所有患者都接受了化疗和放疗。13例患者被诊断为纤维肉瘤(FS)。其中6例在局部复发时进入试验,这6例中有4例再次局部复发,1例同时发生转移。(摘要截短于250字)

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