Distribution and immunoreactivity of cerebral micro-hamartomas in bilateral acoustic neurofibromatosis (neurofibromatosis 2)
Distribution and immunoreactivity of cerebral micro-hamartomas in bilateral acoustic neurofibromatosis (neurofibromatosis 2)
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双侧听神经纤维瘤病（神经纤维瘤病 2）脑微错构瘤的分布和免疫反应性
DOI：
10.1007/bf00294370
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发表时间：
2004
影响因子：
12.7
通讯作者：
P. Kleihues
中科院分区：
文献类型：
--
作者：
Otmar D. Wiestler;K. Siebenthal;H. Schmitt;W. Feiden;P. Kleihues
双侧听神经纤维瘤病（neurofibromatosis 2，NF 2）占所有神经纤维瘤病的不到10%，表现为双侧听神经鞘瘤、脊神经根多发性神经鞘瘤、脑膜瘤、胶质瘤和错构瘤样CNS病变。我们观察到6例神经纤维瘤病2型患者的大脑皮层和基底节中未成熟神经外胚层细胞的发育不良病灶，范围从偶尔的未成熟发育不良细胞簇到大量融合病变。这些细胞，虽然往往多态性和多核，没有显示有丝分裂活性或肿瘤转化的趋势。为了确定这些病灶的组织发生，用各种胶质细胞、神经元和非神经细胞谱系的抗体进行了广泛的免疫细胞化学反应。除S-100蛋白外，未检测到免疫反应性。S-100在这些病灶中一致表达，无论其大小、位置和多态性程度如何。根据细胞学表现、分布和免疫反应性，我们将这些病灶暂定为神经胶质微错构瘤。虽然我们没有系统地分析患者的中枢神经系统与冯Recklinghausen神经纤维瘤病（神经纤维瘤病1，NF 1），目前的研究强烈表明，这些微错构瘤构成了一个形态学标志的双边听神经纤维瘤病（NF 2）。
SummaryBilateral acoustic neurofibromatosis (neurofibromatosis 2, NF2) accounts for less than 10% of all cases of neurofibromatosis and manifests itself with bilateral acoustic schwannomas, multiple schwannomas of spinal nerve roots, meningiomas, glial tumors and hamartomatous CNS lesions. We have observed dysplastic foci of immature neuroectodermal cells in the cerebral cortex and basal ganglia of six patients afflicted with neurofibromatosis 2, ranging from occasional clusters of immature, dysplastic cells to numerous, confluent lesions. These cells, although often polymorphic and multinuclear did not show mitotic acitivity or a tendency for neoplastic transformation. To determine the histogenesis of these foci, extensive immunocytochemical reactions were carried out with antibodies to a variety of glial, neuronal and nonneural cell lineages. With the exception of S-100 protein, no immunoreactivity was detectable. S-100 was consistently expressed in these foci, irrespective of their size, location, and degree of polymorphism. On the basis of cytological appearance, distribution and immunoreactivity we tentatively designate these foci as glial micro-hamartomas. Although we did not systematically analyze the CNS of patients with von Recklinghausen neurofibromatosis (neurofibromatosis 1, NF1), the present study strongly suggests that these micro-hamartomas constitute a morphological hallmark of bilateral acoustic neurofibromatosis (NF2).
影响因子：
158.5
作者：
MARTUZA, RL;ELDRIDGE, R
通讯作者：
ELDRIDGE, R
影响因子：
56.9
作者：
BARKER, D;WRIGHT, E;SKOLNICK, M
通讯作者：
SKOLNICK, M
化学物质逐步发展癌症的细胞生物化学：G. H. A. Clowes 纪念讲座。
影响因子：
11.2
作者：
Farber,E
通讯作者：
Farber,E