DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR - A SURGICALLY CURABLE TUMOR OF YOUNG-PATIENTS WITH INTRACTABLE PARTIAL SEIZURES - REPORT OF 39 CASES
DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR - A SURGICALLY CURABLE TUMOR OF YOUNG-PATIENTS WITH INTRACTABLE PARTIAL SEIZURES - REPORT OF 39 CASES
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DOI：
10.1227/00006123-198811000-00002
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发表时间：
1988-11-01
期刊：
影响因子：
4.8
通讯作者：
VEDRENNE, C
中科院分区：
文献类型：
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作者：
DAUMASDUPORT, C;SCHEITHAUER, BW;VEDRENNE, C
本报告涉及 39 例形态独特且可手术治愈的神经上皮肿瘤病例的临床病理学特征，这些肿瘤与医学部分复杂性癫痫发作相关。所有这些都是幕上的，其特征在于皮质内位置、多结节结构和细胞组成的异质性。组成细胞包括星形胶质细胞、少突胶质细胞和神经元。由于神经元异型性通常不明显，因此肿瘤表面上类似于混合性少突星形细胞瘤。术语“胚胎发育不良神经上皮肿瘤”（DNT）是针对这些独特的病变提出的，其临床病理学特征表明胚胎发育不良起源。除2例患者出现头痛外，部分复杂性癫痫发作是唯一症状。出现症状的年龄为 1 至 19 岁（平均 9 岁）。除了癫痫发作的慢性性质（范围为 2 至 18 年；平均为 9 年）外，三分之一的患者还表现出放射学特征，例如局灶性颅骨畸形，表明肿瘤发病较早且长期存在。在大多数情况下，计算机断层扫描显示“假性囊性”、界限清楚的低密度外观，在某些情况下与局灶对比增强 (18%) 或钙化高密度 (23%) 相关。肿瘤累及颞叶 24 例（62%），额叶 12 例（31%），顶叶和/或枕叶 3 例。尽管 17 名患者 (44%) 的肿瘤切除被认为不完全或部分切除，但长期随访（范围 1 至 18 年；平均 9 年）未显示任何患者出现复发的临床或放射学证据。将13名接受术后放疗的受试者与26名未接受术后放疗的受试者的生存数据进行比较，表明放疗没有明显的益处。 DNT 的鉴定具有治疗和预后意义，因为可以避免积极治疗，从而使这些年轻患者免受放射或化疗的长期有害影响。
This report concerns the clinicopathological features of 39 cases of a morphologically unique and surgically curable group of neuroepithelial tumors associated with medically partial complex seizures. All were supratentorial and characterized by intracortical location, multinodular architecture, and heterogeneity in cellular composition. The constituent cells included astrocytes, oligodendrocytes, and neurons. Because neuronal atypia was often inapparent, the tumors superficially resembled mixed oligoastrocytomas. The term "dysembryoplastic neuroepithelial tumor" (DNT) is proposed for these distinctive lesions the clinicopathological features of which suggest a dysembryoplastic origin. With the exception of the occurrence of headaches in 2 patients, partial complex seizures were the exclusive symptom. Age at onset of symptoms ranged from 1 to 19 years (mean 9 years). In addition to the chronic nature of the seizures (range, 2 to 18 years; mean, 9 years), one-third of the patients showed radiological features, such as focal cranial deformity, indicating that the tumors had an early onset and were of long standing. In most cases, computed tomography showed a "pseudocystic," well-demarcated, low density appearance associated in some cases with focal contrast enhancemnt (18%) or calcific hyperdensity (23%). The tumor involved the temporal lobe in 24 patients (62%), the frontal lobe in 12 (31%), and the parietal and/or occipital lobe in 3 cases. Although tumor removal was considered incomplete or subtotal in 17 patients (44%), long term follow-up (range, 1 to 18 years; mean, 9 years) showed neither clinical nor radiological evidence of recurrence in any patient. Comparison of the survival data of the 13 subjects who had undergone postoperative radiotherapy with 26 who had not indicated that the radiation therapy was of no obvious benefit. The identification of DNT has therapeutic and prognostic implications because aggressive therapy can be avoided, thus sparing these young patients with deleterious long term effects of radio- or chemotherapy.