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透明隔腔的胶样神经元-神经胶质肿瘤伴向侧脑室脑室内扩展:伴有遗传学分析的罕见病例。

Rosette-forming glioneuronal tumor of the septum pellucidum with extension to the supratentorial ventricles: rare case with genetic analysis.

机构信息

Department of Neuropathology, Institute of Neurology and Huashan Hospital of Fudan University, Shanghai, China.

出版信息

Neuropathology. 2012 Jun;32(3):301-5. doi: 10.1111/j.1440-1789.2011.01261.x. Epub 2011 Oct 24.

DOI:10.1111/j.1440-1789.2011.01261.x
PMID:22017246
Abstract

In the original description, rosette-forming glioneuronal tumors (RGNTs) were restricted to the fourth ventricle and/or posterior fossa. Here, we first report an unusual case of RGNT centered in the septum pellucidum and associated with multiple masses occupying the wall of the bilateral lateral ventricles and the third ventricle. No mass was found in the fourth ventricle. Histological and immunohistochemical examination revealed that the tumor presented biphasic differentiation characterized by predominantly neurocytic rosettes and pilocytic astrocytoma-like components with obvious microvascular proliferation. Chromosome 1p/19q deletions and isocitrate dehydrogenase 1 and 2 (IDH1/2) mutations were not identified. Because this case exhibited a worrisome growth pattern, further studies and long-term follow-up are needed to determine the true nature of these tumors.

摘要

在最初的描述中,玫瑰花结形成的神经胶质神经元肿瘤(RGNTs)局限于第四脑室和/或后颅窝。在这里,我们首次报告了一个位于透明隔并伴有多个占据双侧侧脑室和第三脑室壁的肿块的不常见 RGNT 病例。第四脑室没有发现肿块。组织学和免疫组织化学检查显示,肿瘤具有双相分化特征,主要为神经细胞性玫瑰花结和毛细胞型星形细胞瘤样成分,并伴有明显的微血管增生。染色体 1p/19q 缺失和异柠檬酸脱氢酶 1 和 2(IDH1/2)突变未被发现。由于该病例表现出令人担忧的生长模式,需要进一步研究和长期随访来确定这些肿瘤的真正性质。

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