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根据影像学和切除策略评估手术治疗儿童神经节细胞瘤和胚胎发育不良性神经上皮瘤的癫痫转归。

Seizure outcome in surgically treated pediatric gangliogliomas and dysembryoplastic neuroepitheliomas according to imaging and resection strategies.

机构信息

Neurosurgical Department, MedUniWien, Vienna, Austria.

Neurosurgical Department, MedUniWien, Vienna, Austria.

出版信息

Seizure. 2024 Nov;122:19-25. doi: 10.1016/j.seizure.2024.09.002. Epub 2024 Sep 12.

Abstract

PURPOSE

Imaging and resection strategies for pediatric gangliogliomas (GG) and dysembryoplastic neuroepitheliomas (DNET) presenting with epilepsy were retrospectively analyzed in a consecutive institutional series of surgically treated patients.

METHODS

Twenty-two children (median 8 years, 3-18 years) presented with seizures for 30 months median (14-55.2 months) due to a histologically verified GG/DNET.

RESULTS

There were 20 GG and 2 DNT, 68 % located temporal, 32 % extra-temporal. Seizure history was significantly longer in temporal cases (38 versus 14 months median, p < 0.01). MRI contrast enhancement was present in 50 % and methionine (MET) positron emission tomography (PET) uptake in 70 % (standard uptake values (SUVs) 2.92 mean, from 1.6 to 6.4). 27 % had glucose PET hypometabolism. Primarily, in temporal GG, ECoG (electrocorticography) -guided lesionectomies were performed in 87 % and antero-mesial temporal lobe resections (AMTLR) in 13 %, whereas in extra-temporal GG/DNETs, lesionectomies were performed in 100 %. ILAE Class 1 seizure outcome was primarily achieved in 73 % of the temporal cases, and was increased to 93 % by performing six repeat surgeries using AMTLR. Extratemporal patients experienced ILAE Class 1 seizure outcomes in 86 % without additional surgeries, although harboring significantly more residual tumor (p < 0.005, mean follow-up 28 months).

CONCLUSION

In children, MET PET imaging for suspected GG is proposed preoperatively showing a high diagnostic sensitivity and an option to delineate the lesions for navigated resection, whereas MRI contrast behavior was of no differential diagnostic use. As a surgical strategy we propose primarily lesionectomies for extratemporal but AMTLR for temporal GG respecting eloquent brain areas.

摘要

目的

回顾性分析连续机构系列手术治疗的患儿中,表现为癫痫的神经节细胞瘤(GG)和胚胎发育不良性神经上皮瘤(DNET)的影像学和切除策略。

方法

22 名儿童(中位年龄 8 岁,3-18 岁)因组织学证实的 GG/DNET 出现癫痫,中位癫痫病史 30 个月(14-55.2 个月)。

结果

20 例为 GG,2 例为 DNT,68%位于颞叶,32%位于颞叶以外。颞叶病例的癫痫病史明显更长(38 个月与 14 个月中位数,p<0.01)。50%的 MRI 对比增强,70%的蛋氨酸(MET)正电子发射断层扫描(PET)摄取(标准摄取值(SUVs)2.92,1.6-6.4)。27%的葡萄糖 PET 呈低代谢。主要在颞叶 GG 中,87%的患者行 ECoG(脑电图)引导的病变切除术,13%的患者行前内侧颞叶切除术(AMTLR),而在颞叶以外的 GG/DNET 中,100%的患者行病变切除术。73%的颞叶病例主要达到国际抗癫痫联盟(ILAE)1 级癫痫发作结局,通过 6 次重复使用 AMTLR 进行手术,增加到 93%。无额外手术的颞叶以外患者 ILAE 1 级癫痫发作结局发生率为 86%,但肿瘤残留明显更多(p<0.005,中位随访 28 个月)。

结论

在儿童中,对于疑似 GG,MET PET 成像术前建议使用,具有较高的诊断敏感性,并为导航切除勾画病变提供了一种选择,而 MRI 对比行为在鉴别诊断方面没有作用。作为一种手术策略,我们建议主要对颞叶以外的病变进行切除术,但对颞叶 GG 进行 AMTLR,以尊重功能区。

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