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IDH1/2 野生型 2 级和 3 级胶质瘤伴分子胶质母细胞瘤样特征的癫痫发作过程与 IDH1/2 野生型胶质母细胞瘤明显不同。

IDH1/2 wildtype gliomas grade 2 and 3 with molecular glioblastoma-like profile have a distinct course of epilepsy compared to IDH1/2 wildtype glioblastomas.

机构信息

Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands.

Department of Neurosurgery, Haaglanden Medical Center, The Hague, the Netherlands.

出版信息

Neuro Oncol. 2023 Apr 6;25(4):701-709. doi: 10.1093/neuonc/noac197.

DOI:10.1093/neuonc/noac197
PMID:35972438
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10076940/
Abstract

BACKGROUND

IDH1/2 wildtype (IDHwt) glioma WHO grade 2 and 3 patients with pTERT mutation and/or EGFR amplification and/or + 7/-10 chromosome gain/loss have a similar overall survival time as IDHwt glioblastoma patients, and are both considered glioblastoma IDHwt according to the WHO 2021 classification. However, differences in seizure onset have been observed. This study aimed to compare the course of epilepsy in the 2 glioblastoma subtypes.

METHODS

We analyzed epilepsy data of an existing cohort including IDHwt histologically lower-grade glioma WHO grade 2 and 3 with molecular glioblastoma-like profile (IDHwt hLGG) and IDHwt glioblastoma patients. Primary outcome was the incidence proportion of epilepsy during the disease course. Secondary outcomes included, among others, onset of epilepsy, number of seizure days, and antiepileptic drug (AED) polytherapy.

RESULTS

Out of 254 patients, 78% (50/64) IDHwt hLGG and 68% (129/190) IDHwt glioblastoma patients developed epilepsy during the disease (P = .121). Epilepsy onset before histopathological diagnosis occurred more frequently in IDHwt hLGG compared to IDHwt glioblastoma patients (90% vs 60%, P < .001), with a significantly longer median time to diagnosis (3.5 vs 1.3 months, P < .001). Median total seizure days was also longer for IDHwt hLGG patients (7.0 vs 3.0, P = .005), and they received more often AED polytherapy (32% vs 17%, P = .028).

CONCLUSIONS

Although the incidence proportion of epilepsy during the entire disease course is similar, IDHwt hLGG patients show a significantly higher incidence of epilepsy before diagnosis and a significantly longer median time between first seizure and diagnosis compared to IDHwt glioblastoma patients, indicating a distinct clinical course.

摘要

背景

IDH1/2 野生型(IDHwt)胶质瘤 WHO 分级 2 级和 3 级患者存在 pTERT 突变和/或 EGFR 扩增和/或+7/-10 染色体获得/缺失,其总生存时间与 IDHwt 胶质母细胞瘤患者相似,根据 WHO 2021 分类,两者均被认为是 IDHwt 胶质母细胞瘤。然而,癫痫发作的起始存在差异。本研究旨在比较两种胶质母细胞瘤亚型的癫痫发作过程。

方法

我们分析了包括 IDHwt 组织学低级别胶质瘤 WHO 分级 2 级和 3 级且分子具有胶质母细胞瘤样特征(IDHwt hLGG)和 IDHwt 胶质母细胞瘤患者在内的现有队列中的癫痫发作数据。主要结局是疾病过程中癫痫发作的发生率比例。次要结局包括癫痫发作的起始时间、癫痫发作天数和抗癫痫药物(AED)联合治疗等。

结果

在 254 例患者中,64%(50/64)的 IDHwt hLGG 和 68%(129/190)的 IDHwt 胶质母细胞瘤患者在疾病过程中出现癫痫发作(P=0.121)。与 IDHwt 胶质母细胞瘤患者相比,IDHwt hLGG 患者更常在组织病理学诊断前出现癫痫发作(90% vs 60%,P<0.001),中位诊断时间明显更长(3.5 个月 vs 1.3 个月,P<0.001)。IDHwt hLGG 患者的总癫痫发作天数也更长(7.0 天 vs 3.0 天,P=0.005),并且更常接受 AED 联合治疗(32% vs 17%,P=0.028)。

结论

尽管整个疾病过程中癫痫发作的发生率比例相似,但与 IDHwt 胶质母细胞瘤患者相比,IDHwt hLGG 患者在诊断前的癫痫发作发生率更高,且首次癫痫发作和诊断之间的中位时间明显更长,提示其具有明显不同的临床病程。

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