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一种具有横纹肌样特征的罕见星形细胞瘤。

A rare astrocytic tumor with rhabdoid features.

作者信息

Nagai Shoichi, Kurimoto Masanori, Ishizawa Shin, Hayashi Nakamasa, Hamada Hideo, Kamiyama Hironaga, Endo Shunro

机构信息

Department of Neurosurgery, Faculty of Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan.

出版信息

Brain Tumor Pathol. 2009;26(1):19-24. doi: 10.1007/s10014-008-0241-9. Epub 2009 Apr 30.

DOI:10.1007/s10014-008-0241-9
PMID:19408093
Abstract

We report an extremely rare tumor presenting with rhabdoid features in the left temporoparietal lobe near the trigone in an 18-year-old Japanese man. This tumor mainly consisted of medium to large round cells that proliferated diffusely and incoherently with a scant extracellular matrix. These tumor cells had an eccentric nucleus and an eosinophilic cytoplasm containing inclusion bodies and bundles of intermediate filaments. The nuclei of these cells were vesicular with prominent nucleoli. This tumor had an area appearing to be diffuse astrocytoma peripherally and lacked a primitive neuroectodermal tumor component, a mesenchymal component, and epithelial differentiation. INI expression, which is not observed in atypical teratoid/ rhabdoid tumor (AT/RT), was found in this tumor. From these findings, we concluded that this tumor was not AT/RT but an astrocytic tumor with rhabdoid features. We also concluded that the tumor cells exhibiting rhabdoid features had secondarily arisen from the peripheral area presenting an appearance of diffuse astrocytoma.

摘要

我们报告了一名18岁日本男性,其左颞顶叶靠近三角区处出现了一种具有横纹肌样特征的极其罕见的肿瘤。该肿瘤主要由中等至大的圆形细胞组成,这些细胞弥漫性且无序地增殖,细胞外基质稀少。这些肿瘤细胞有一个偏心核和一个嗜酸性细胞质,其中含有包涵体和中间丝束。这些细胞的核呈泡状,核仁突出。该肿瘤周边有一个区域看似弥漫性星形细胞瘤,且缺乏原始神经外胚层肿瘤成分、间充质成分和上皮分化。在该肿瘤中发现了INI表达,而在非典型畸胎样/横纹肌样肿瘤(AT/RT)中未观察到这种表达。基于这些发现,我们得出结论,该肿瘤不是AT/RT,而是一种具有横纹肌样特征的星形细胞瘤。我们还得出结论,表现出横纹肌样特征的肿瘤细胞继发于呈现弥漫性星形细胞瘤外观的周边区域。

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