Intractable epilepsy due to a rosette-forming glioneuronal tumor with a dysembryoplastic neuroepithelial background.
Intractable epilepsy due to a rosette-forming glioneuronal tumor with a dysembryoplastic neuroepithelial background.
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由于具有胚胎发育异常的神经上皮背景的玫瑰花状胶质神经元肿瘤引起的难治性癫痫。
DOI：
10.1111/neup.12450
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发表时间：
2017
期刊：
影响因子：
2.3
通讯作者：
Sasaki M.
中科院分区：
文献类型：
--
作者：
Sumitomo N;Ishiyama A;Shibuya M;Nakagawa E;Kaneko Y;Takahashi A;Otsuki T;Kakita A;Saito Y;Sato N;Sugai K;Sasaki M.
玫瑰花结形成胶质神经元肿瘤（RGNT）最初被报道为幕下肿瘤，包括小神经细胞玫瑰花结和星形细胞成分。然而，少数研究报告了幕上RGNT出现在大脑半球。在这里，我们报告了一个不寻常的情况下，涉及一个9岁的男孩与幕上RGNT谁提出了顽固性癫痫和行为改变。脑部MRI显示右侧顶叶内侧下叶存在边界清楚的占位性病变伴分隔。脑电图显示右额叶、颞叶和顶叶区域多灶性棘波。肿瘤切除术后癫痫发作频率显著降低。组织学检查发现，显着的神经细胞玫瑰花结形成出现与特定的胶质神经元元素的胚胎发育不良性神经上皮肿瘤（DNT）。虽然发病机制尚未阐明，一个幕上RGNT提出癫痫可能会表现出玫瑰花结的组成部分，这是这种肿瘤的主要特征，对特定的胶质神经元模拟DNT的元素的背景。然而，RGNT发生在第四脑室以外的区域是罕见的，并且由于RGNT引起的癫痫的发病机制尚未完全阐明。需要进一步的临床和组织学研究来了解RGNT引起癫痫的病理学基础。
A rosette‐forming glioneuronal tumor (RGNT) was initially reported as an infratentorial tumor that comprised both small neurocytic rosettes and astrocytic components. However, a few studies have reported supratentorial RGNTs arising in the cerebral hemispheres. Here, we report an unusual case involving a 9‐year‐old boy with a supratentorial RGNT who presented with intractable epilepsy and behavioral changes. Brain MRI revealed a well‐circumscribed space‐occupying lesion with septae in the right inferomedial parietal lobe. Electroencephalography showed multifocal spikes over the right frontal, temporal and parietal regions. The seizure frequency decreased dramatically after tumorectomy. Histopathological examination revealed prominent neurocytic rosette formation appearing with the specific glioneuronal element of a dysembryoplastic neuroepithelial tumor (DNT). Although the pathogenesis has not been elucidated, a supratentorial RGNT presenting with epilepsy may exhibit a rosette component, which is the major feature of this tumor, against the background of a specific glioneuronal element mimicking DNT. However, RGNT arising in regions other than the fourth ventricle is rare, and the pathogenesis of epilepsy due to RGNT has not been fully elucidated. Further clinical and histological studies are required to understand the pathology underlying epilepsy caused by RGNT.