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脑胚胎发育不良性神经上皮肿瘤患儿的癫痫发作——多项研究的手术结果综述

Seizures in children with dysembryoplastic neuroepithelial tumors of the brain--A review of surgical outcomes across several studies.

作者信息

Ranger Adrianna, Diosy David

机构信息

Department of Clinical Neurological Sciences, Division of Neurosurgery (Pediatric Neurosurgery), Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada,

出版信息

Childs Nerv Syst. 2015 Jun;31(6):847-55. doi: 10.1007/s00381-015-2675-9. Epub 2015 Mar 21.

Abstract

PURPOSE

In children and adolescents, dysembryoplastic neuroepithelial tumors (DNETs) of the brain present with seizures almost 100% of the time, potentially creating significant long-term morbidity and disability despite the generally indolent course of the lesion. These tumors also tend to be quite resistant to anti-epileptic drugs which, themselves, can be associated with long-term side effects and resultant disability. Many clinicians advocate early surgical resection of these lesions, but how effective this approach is, and how aggressive tumor removal should be, continues to be debated.

METHODS

We performed a systematic review of the relevant literature to identify all reports of DNET resections in pediatric patients published over the past 20 years. In all, over 3000 MEDLINE abstracts were reviewed, ultimately resulting in 13 studies with 185 pediatric DNET patients to review.

RESULTS

Surgical resection of the lesion was effective at improving seizures in over 98% of patients and at achieving long-term seizure freedom in 86%. Surgical resection of DNETs also appeared to be quite safe, with no reported perioperative deaths and an overall rate of postoperative complications of 12%; the vast majority of these complications were transient.

CONCLUSIONS

Total gross resection of the lesion was the only factor statistically correlated with long-term seizure freedom (r = 0.63, p = 0.03). However, data remain lacking regarding whether this translates into more extensive procedures-like brain mapping and partial lobectomies-being any more effective than simple lesionectomies alone. Further research is clearly needed to address this and other crucial questions.

摘要

目的

在儿童和青少年中,脑胚胎发育不良性神经上皮肿瘤(DNETs)几乎100%会出现癫痫发作,尽管该病变通常病程进展缓慢,但仍可能造成严重的长期发病和残疾。这些肿瘤往往对抗癫痫药物有相当的耐药性,而抗癫痫药物本身也可能伴有长期副作用及由此导致的残疾。许多临床医生主张早期手术切除这些病变,但这种方法的效果如何,以及肿瘤切除应激进到何种程度,仍存在争议。

方法

我们对相关文献进行了系统回顾,以确定过去20年发表的所有关于儿科患者DNETs切除术的报告。总共查阅了3000多篇MEDLINE摘要,最终得到13项研究,涉及185例儿科DNET患者以供审查。

结果

病变的手术切除对超过98%的患者改善癫痫发作有效,86%的患者实现了长期无癫痫发作。DNETs的手术切除似乎也相当安全,未报告围手术期死亡,术后并发症总发生率为12%;这些并发症绝大多数是短暂的。

结论

病变的全瘤切除是与长期无癫痫发作唯一有统计学相关性的因素(r = 0.63,p = 0.03)。然而,关于这是否转化为更广泛的手术——如脑图谱绘制和部分肺叶切除术——比单纯的病变切除术更有效,仍然缺乏数据。显然需要进一步的研究来解决这个问题和其他关键问题。

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本文引用的文献

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Malignant transformation of DNETs: a case report and literature review.
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