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首页> 外文期刊>Journal of neurology >Prolonged survival in adult neurofibromatosis type i patients with recurrent high-grade gliomas treated with bevacizumab
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Prolonged survival in adult neurofibromatosis type i patients with recurrent high-grade gliomas treated with bevacizumab

机译：贝伐单抗治疗的成人I型成人神经纤维瘤病复发性高级神经胶质瘤患者的生存期延长

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Astrocytic tumors, especially optic pathway pilocytic astrocytomas, are common in pediatric NF1 patients. High-grade gliomas (HGGs) appear to be rare in adult and pediatric NF1 patients. This is a series of five consecutive, adult NF1 patients with recurrent HGGs treated at The University of Texas MD Anderson Cancer Center. Four patients met consensus clinical criteria for NF1 and one patient had presumed segmental NF1. Three patients had glioblastomas, one gliosarcoma, and one progressive, enhancing optic pathway glioma which was not biopsied. Two tumors had molecular testing performed; both were IDH wild type and activating oncogene mutations (1 BRAFV600E and 1 PIK3CA mutation) were found in these tumors. All five patients received bevacizumab-containing regimens at tumor recurrence. The median number of 4-week cycles of bevacizumab was 20. All five patients experienced prolonged post-recurrence survival following bevacizumab treatment ranging from ten to 72 months. The median overall survival from HGG diagnosis was 72.6 months with three patients alive and progression free at last follow-up. Three out of five patients developed vascular complications leading to bevacizumab discontinuation. In this case series, adult NF1 patients with recurrent HGGs had prolonged, post-recurrence survival after treatment with bevacizumab-containing regimens. Based on these results, further study of antiangiogenic therapy in NF1 patients with HGGs and bevacizumab-response in sporadic HGG patients with NF1-mutated tumors is warranted.
机译：小儿NF1患者常见星形细胞肿瘤，特别是视神经纤维细胞星形细胞瘤。在成人和小儿NF1患者中，高度神经胶质瘤（HGG）似乎很少。这是在德克萨斯大学MD安德森癌症中心接受治疗的连续5例连续HGG复发的成年NF1成人患者的系列。 4例患者符合NF1的共识临床标准，其中1例患者推测为节段性NF1。 3例患有胶质母细胞瘤，1例神经胶质肉瘤和1例进行了活检的渐进性增强视神经胶质瘤。对两个肿瘤进行了分子检测。两者均为IDH野生型，并且在这些肿瘤中发现了激活的癌基因突变（1个BRAFV600E和1个PIK3CA突变）。所有五名患者在肿瘤复发时均接受含贝伐单抗治疗。贝伐单抗的4周周期的中位数为20。所有五名患者在接受贝伐单抗治疗后的十至72个月不等，均具有较长的复发后生存期。 HGG诊断的平均总生存期为72.6个月，其中3名患者还活着并且在最后一次随访中无进展。五分之三的患者出现血管并发症，导致贝伐单抗停药。在这个案例系列中，患有复发性HGG的成年NF1患者在使用含贝伐单抗的方案治疗后具有延长的复发后生存期。基于这些结果，有必要进一步研究在患有HGGs的NF1患者中的抗血管生成治疗和在患有NF1突变的散发性HGG患者中的贝伐单抗反应。

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