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嗅鞘细胞瘤伴神经纤维瘤样特征:病例报告及文献复习。

Olfactory ensheathing cell tumor with neurofibroma-like features: a case report and review of the literature.

机构信息

Department of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taiwan, ROC.

出版信息

J Neurooncol. 2010 Mar;97(1):117-22. doi: 10.1007/s11060-009-9986-1. Epub 2009 Aug 19.

Abstract

A 32-year-old man had seizure attack since April 2008 and radiographic examination revealed a heterogeneous enhancing mass at the left subfrontal region. He underwent craniotomy for tumor removal on October 1, 2008. The tumor, which was grayish white with glistening appearance and rubbery consistency, was traced to the proximal part of left olfactory tract. Histopathological examination revealed a hypocellular tumor with dense hyalinization in most areas. The tumor cells had ovoid to elongate and often comma-shaped nucleus. Myxoid change of the stroma was apparent in places. Most of the tumor cells were immuno-reactive for S-100 protein. Staining for Leu 7 (CD57 or HNK-1) was negative. Bodian method illustrated many axons within the tumor. Ultrastructural study of the tumor cells showed features compatible with those of olfactory ensheathing cell. The tumor was designated as olfactory ensheathing cell tumor with neurofibroma-like features. There have been 14 nerve sheath tumors arising from the olfactory nerve reported in the literature; all of them had the morphology of schwannoma. Our case, which had the morphology simulating neurofibroma was the first of its kind to be recorded.

摘要

一位 32 岁男性自 2008 年 4 月起出现癫痫发作,影像学检查显示左额前区有一混杂增强肿块。他于 2008 年 10 月 1 日接受了肿瘤切除术。肿瘤呈灰白色,有光泽,质地橡胶样,起源于左嗅束近端。组织病理学检查显示肿瘤细胞稀疏,大部分区域有致密玻璃样变。肿瘤细胞呈卵圆形至长形,常呈逗号形核。间质黏液样变明显。大多数肿瘤细胞对 S-100 蛋白呈免疫反应阳性,Leu 7(CD57 或 HNK-1)染色阴性。Bodian 法显示肿瘤内有许多轴突。肿瘤细胞的超微结构研究显示与嗅鞘细胞的特征一致。该肿瘤被诊断为具有神经纤维瘤样特征的嗅鞘细胞瘤。文献中已有 14 例发生于嗅神经的神经鞘瘤报道,它们均具有施万细胞瘤的形态。我们的病例形态模拟神经纤维瘤,为首例报道。

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