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
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
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DOI：
10.1093/neuonc/noac197
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发表时间：
2022-09-14
期刊：
影响因子：
15.9
通讯作者：
Koekkoek, Johan A. F.
中科院分区：
文献类型：
--
作者：
van Opijnen, Mark P.;Tesileanu, C. Mircea S.;Koekkoek, Johan A. F.
WHO 2级和3级pTERT突变和/或EGFR扩增和/或+ 7/-10染色体获得/丢失的IDH1/2野生型（IDHwt）胶质瘤患者与IDHwt胶质母细胞瘤患者的总生存时间相似，并且根据WHO 2021分类均被认为是IDHwt胶质母细胞瘤。然而，不同的癫痫发作已被观察到。本研究旨在比较2种胶质母细胞瘤亚型的癫痫病程。方法我们分析了一个现有队列的癫痫数据，包括组织学上较低级别胶质瘤WHO 2级和3级分子胶质母细胞瘤样特征（IDHwt hLGG）和IDHwt胶质母细胞瘤患者。主要观察指标为病程中癫痫的发生率。次要结局包括癫痫发作、发作天数和抗癫痫药物（AED）综合治疗。结果254例患者中，78% (50/64)IDHwt hLGG和68% (129/190)IDHwt胶质母细胞瘤患者在疾病期间发生癫痫（P = 0.121）。与IDHwt胶质母细胞瘤患者相比，IDHwt hLGG患者在组织病理学诊断前癫痫发作的频率更高（90% vs 60%, P < 0.001），中位诊断时间明显更长（3.5 vs 1.3个月，P < 0.001）。IDHwt hLGG患者的中位总癫痫发作天数也更长（7.0 vs 3.0, P = 0.005），并且他们更频繁地接受AED综合治疗（32% vs 17%, P = 0.028）。结论虽然癫痫在整个病程中的发生率相似，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生率明显高于IDHwt，且首次发作至诊断的中位时间明显长于IDHwt胶质母细胞瘤患者，临床病程明显不同。
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.