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[胚胎发育不良性神经上皮肿瘤]

[Dysembryoplastic neuroepithelial tumor].

作者信息

Vajtai I, Varga Z, Bodosi M, Kopniczky Z, Kóbor J, Vörös E

机构信息

Szent-Györgyi Albert Orvostudományi Egyetem, Pathologiai Intézet, Szeged.

出版信息

Orv Hetil. 1995 Nov 26;136(48):2623-7.

PMID:8539063
Abstract

The dysembryoplastic neuroepithelial tumor is a benign central nervous system neoplasm of children and young adults manifesting almost exclusively in complex partial seizures. The authors report the case of an 8-year-old boy presenting with characteristic clinical and radiologic features who subsequently underwent surgery. Light microscopic, immunohistochemical and ultrastructural traits of the tumor are described demonstrating pluripotential differentiation of tumor cells and architectural features suggesting a dysontogenic lesion. A brief literature review on the biology and histologic diagnosis of the dysembryoplastic neuroepithelial tumor is provided. Although this uncommon tumor accounts for only a minority of intracranial neoplasms, its pathogenetic role has to be considered in the differential diagnosis of temporal lobe epilepsies. This is the first report on this recently described, rare neoplastic condition in Hungary.

摘要

胚胎发育不良性神经上皮肿瘤是儿童和青年的一种良性中枢神经系统肿瘤,几乎仅表现为复杂部分性癫痫发作。作者报告了一例8岁男孩,其具有特征性的临床和放射学表现,随后接受了手术。描述了肿瘤的光镜、免疫组化和超微结构特征,显示肿瘤细胞具有多能分化以及提示发育异常性病变的结构特征。提供了关于胚胎发育不良性神经上皮肿瘤生物学和组织学诊断的简要文献综述。尽管这种罕见肿瘤仅占颅内肿瘤的少数,但在颞叶癫痫的鉴别诊断中必须考虑其致病作用。这是匈牙利关于这种最近描述的罕见肿瘤性疾病的首例报告。

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