Pediatric malignant peripheral nerve sheath tumor: The Italian and German soft tissue sarcoma cooperative group
Pediatric malignant peripheral nerve sheath tumor: The Italian and German soft tissue sarcoma cooperative group
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DOI：
10.1200/jco.2005.01.4886
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发表时间：
2005-11-20
影响因子：
45.3
通讯作者：
Treuner, J
中科院分区：
文献类型：
--
作者：
Carli, M;Ferrari, A;Treuner, J
目的评估儿童恶性外周神经鞘瘤（MPNSTs）的化疗和放疗的价值，并确定与outcome.Patients和方法相关的危险因素，共有167名未经治疗的符合条件的患者参加了意大利和德国的研究在1975年和1998年之间进入本分析。17%的患者患有1型神经纤维瘤病（NF 1）。结果中位随访7年，5年总生存率（OS）和无进展生存率（PFS）分别为51%和37%。组间横纹肌肉瘤研究（IRS）分组的5年OS和PFS如下：组I，分别为82%和61%;组II，分别为62%和37%;组III，分别为32%和27%;组IV，分别为26%和21%。单因素分析确定IRS组，大小，侵袭性，原发部位，年龄和存在的NF 1作为预后因素;多因素分析确定没有NF 1，肿瘤侵袭性T1，IRS组I至II和原发部位的四肢作为独立的有利因素OS。结论MPNST是一种侵袭性肿瘤，手术切除是治疗成功的关键。术后放射治疗可能在改善局部控制的作用，在微小残留肿瘤的患者。报告的对原发性化疗的反应表明，它可能是有效的，在诊断时被认为是不可切除的肿瘤患者。
Purpose To assess the value of chemotherapy and radiotherapy in children with malignant peripheral nerve sheath tumors (MPNSTs) and to identify risk factors associated with outcome.Patients and Methods A total of 167 untreated eligible patients enrolled onto the Italian and German studies between 1975 and 1998 entered this analysis. Seventeen percent of patients had neurofibromatosis type 1 (NF1). Chemotherapy was administered to 74% of patients; radiotherapy was administered to 38% of patients.Results With a median follow-up of 7 years, 5-year overall survival (OS) and progression-free survival (PFS) were 51% and 37%, respectively. The 5-year OS and PFS by Intergroup Rhabdomyosarcoma Study (IRS) groupings were as follows: group I, 82% and 61 %; group II, 62% and 37%; group III, 32% and 27%; group IV, 26% and 21%, respectively. Univariate analysis identified IRS groups, size, invasiveness, primary site, age, and presence of NF1 as prognostic factors; multivariate analysis identified absence of NF1, tumor invasiveness T1, IRS groups I to II and extremity of primary site as independent favorable factors for OS. A trend was observed toward a benefit from radiotherapy after initial gross resection. The overall response rate to primary chemotherapy, including minor responses, in group III patients was 45%.Conclusion MPNST is an aggressive tumor for which complete surgical resection is the mainstay of successful treatment. Postoperative radiotherapy may have a role in improving local control in patients with minimal residual tumor. The reported responses to primary chemotherapy suggest that it may be effective in patients with tumor considered unresectable at diagnosis.