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长期癫痫相关肿瘤。

Long-term epilepsy-associated tumors.

机构信息

Department of Clinical and Experimental Epilepsy, UCL, Institute of Neurology, Queen Square, London, UK.

出版信息

Brain Pathol. 2012 May;22(3):350-79. doi: 10.1111/j.1750-3639.2012.00582.x.

Abstract

The term long-term epilepsy associated tumor (LEAT) encompasses lesions identified in patients investigated for long histories (often 2 years or more) of drug-resistant epilepsy. They are generally slowly growing, low grade, cortically based tumors, more often arising in younger age groups and in many cases exhibit neuronal in addition to glial differentiation. Gangliogliomas and dysembryoplastic neuroepithelial tumors predominate in this group. LEATs are further united by cyto-architectural changes that may be present in the adjacent cortex which have some similarities to developmental focal cortical dysplasias (FCD); these are now grouped as FCD type IIIb in the updated International League Against Epilepsy (ILAE) classification. In the majority of cases, surgical treatments are beneficial from both perspectives of managing the seizures and the tumor. However, in a minority, seizures may recur, tumors may show regrowth or recurrence, and rarely undergo anaplastic progression. Predicting and identifying tumors likely to behave less favorably are key objectives of the neuropathologist. With immunohistochemistry and modern molecular pathology, it is becoming increasingly possible to refine diagnostic groups. Despite this, some LEATs remain difficult to classify, particularly tumors with "non-specific" or diffuse growth patterns. Modification of LEAT classification is inevitable with the goal of unifying terminological criteria applied between centers for accurate clinico-pathological-molecular correlative data to emerge. Finally, establishing the epileptogenic components of LEAT, either within the lesion or perilesional cortex, will elucidate the cellular mechanisms of epileptogenesis, which in turn will guide optimal surgical management of these lesions.

摘要

长期癫痫相关肿瘤(LEAT)一词涵盖了在抗药性癫痫病史较长(通常 2 年或更长时间)的患者中发现的病变。它们通常生长缓慢,级别较低,位于皮质下,更多见于年轻年龄段,并且在许多情况下表现出神经元和神经胶质分化。神经节胶质瘤和发育不良性神经上皮肿瘤在这一组中更为常见。LEAT 还具有细胞结构改变,这些改变可能存在于相邻皮质中,与发育性局灶性皮质发育不良(FCD)有一些相似之处;在更新的国际抗癫痫联盟(ILAE)分类中,这些改变现在被归类为 FCD Ⅲb 型。在大多数情况下,手术治疗从控制癫痫发作和肿瘤两个方面都有益处。然而,在少数情况下,癫痫可能会复发,肿瘤可能会出现复发或进展,极少数情况下会发生间变进展。预测和识别可能表现不佳的肿瘤是神经病理学家的主要目标。随着免疫组织化学和现代分子病理学的发展,越来越有可能对诊断组进行细化。尽管如此,一些 LEAT 仍然难以分类,特别是具有“非特异性”或弥漫性生长模式的肿瘤。LEAT 分类的修改是不可避免的,目的是统一应用于各中心的术语标准,以获得准确的临床病理分子相关性数据。最后,确定 LEAT 病变内或病变周围皮质的致痫成分,将阐明致痫发生的细胞机制,这反过来又将指导这些病变的最佳手术治疗。

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