A cortical dysembryoplastic neuroepithelial tumor initially occurring in the periventricular white matter
A cortical dysembryoplastic neuroepithelial tumor initially occurring in the periventricular white matter
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DOI：
10.3171/2010.10.peds10391
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发表时间：
2010-12-01
影响因子：
1.9
通讯作者：
Kinouchi, Hiroyuki
中科院分区：
文献类型：
--
作者：
Kawataki, Tomoyuki;Sato, Eiji;Kinouchi, Hiroyuki
本文报告一例罕见的胚胎发育不良神经上皮性肿瘤(DNet)，最初表现为侧脑室附近伴有钙化的小白质病变，7年后延伸至额叶皮质。这名1岁男孩最初部分癫痫发作，最初CT显示左侧额叶深层白质中有一个小的低密度区，周围有一个微小的钙化肿块。7年后，他的癫痫发作对抗癫痫药物已变得难以治疗。MR成像显示一个较大的肿块，从钙化的病变一直延伸到邻近的皮质表面，他接受了手术，肿瘤被次全切除。组织学检查证实，它是一个由黏液背景中漂浮的神经元组成的小少突胶质细胞簇组成的dNet。此例的肿瘤进展模式表明，皮质中的dNet起源于靠近脑室的室管膜下生发层(DOI 10.3171/2010 10。PEDS 10391)
In this report a rare case of dysembryoplastic neuroepithelial tumor (DNET) initially presented as a small white matter lesion with calcification adjacent to the lateral ventricle and extending to the frontal cortex after 7 years This 1-year-old boy initially suffered from partial seizures Initial CT revealed a small low-density area surrounding a tiny calcified mass in the deep white matter of the left frontal lobe Seven years later, his seizures had become intractable to antiepileptic agents, and MR imaging demonstrated a relatively large mass extending from the calcified lesion up to the adjacent cortical surface He underwent surgery and the tumor was subtotally removed Histological examination of the tumor verified it as a DNET consisting of clusters of small oligodendrocytes with floating neurons in the mucoid background The pattern of the tumor progression in this case suggests that a DNET in the cortex originates from the subependymal germinal layer near the ventricle (DOI 10.3171/2010.10.PEDS10391)