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[胚胎发育不良性神经上皮肿瘤(DNT)的临床病理特征:4例报告]

[Clinicopathological features of dysembryoplastic neuroepithelial tumor (DNT): four cases report].

作者信息

Hashizume K, Tanaka T, Daita G, Yonemasu Y, Miyamoto M, Miyokawa N, Wakai S, Fukushima H

机构信息

Department of Neurosurgery, Asahikawa Medical College.

出版信息

No Shinkei Geka. 1994 Aug;22(8):743-8.

PMID:8072632
Abstract

We reported four cases of dysembryoplastic neuroepithelial tumor (DNT) with intractable complex partial seizures, and analyzed their clinical and pathological features. The age of patients ranged from four to nineteen years old. Three were boys and one was a girl. The age of seizure onset ranged from eight months to five years. They did not have any neurological deficits. In all cases, CT scan revealed a low density mass in the temporal lobe. Two cases demonstrated calcifications, and another two cysts. The mass demonstrated low signal intensity in T-1 weighted MR images with mild contrast enhancement by Gd-DTPA. Long-term video-EEG monitoring revealed the epileptogenic focus in the temporal lobe on the side of the mass lesion. Anterior temporal lobectomy with total removal of the tumor was carried out in all cases. Hippocampectomy was performed in three cases in which intraoperative electrocorticogram demonstrated spike discharges in the hippocampus. In the surgical specimens, the tumor consisted of increased astrocytes, oligodendrocytes, and some neurons. A cortical nodule appeared in one case, and cortical dysplasia appeared in two cases. It was difficult to distinguish the tumor of case 3 from ganglioglioma because of mild neuronal atypia. The tumor of case 4 demonstrated increased capillary vessels and was also difficult to differentiate from vascular anomalies. The pathological diagnosis of DNT was still difficult. Consequently, we speculated that DNT included some transitional types between congenital anomalies and ganglioglioma.

摘要

我们报告了4例伴有难治性复杂部分性发作的胚胎发育不良性神经上皮肿瘤(DNT),并分析了其临床和病理特征。患者年龄在4岁至19岁之间。3例为男性,1例为女性。癫痫发作起始年龄在8个月至5岁之间。他们均无任何神经功能缺损。所有病例中,CT扫描显示颞叶有低密度肿块。2例有钙化,另2例有囊肿。该肿块在T1加权磁共振图像上呈低信号强度,经钆喷酸葡胺(Gd-DTPA)轻度强化。长期视频脑电图监测显示癫痫病灶位于肿块病变侧的颞叶。所有病例均行前颞叶切除术并完整切除肿瘤。3例术中皮层脑电图显示海马有棘波放电,行海马切除术。手术标本中,肿瘤由增多的星形胶质细胞、少突胶质细胞和一些神经元组成。1例出现皮质结节,2例出现皮质发育异常。病例3的肿瘤因有轻度神经元异型性,难以与节细胞胶质瘤区分。病例4的肿瘤毛细血管增多,也难以与血管畸形区分。DNT的病理诊断仍很困难。因此,我们推测DNT包括一些先天性异常与节细胞胶质瘤之间的过渡类型。

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