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具有间变特征的脑室多形性黄色星形细胞瘤。

Intraventricular pleomorphic xanthoastrocytoma with anaplastic features.

机构信息

Department of Pathology, Brain Research Institute, University of Niigata, Niigata, Japan.

出版信息

Neuropathology. 2010 Aug;30(4):443-8. doi: 10.1111/j.1440-1789.2009.01080.x. Epub 2009 Dec 28.

Abstract

Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic tumor that usually occurs in the superficial cerebral hemispheres of children and young adults and has a relatively favorable prognosis. We report an unusual case of supratentorial, intraventricular tumor in a 52-year-old man. The tumor was composed of pleomorphic cells, including giant cells, most of which were multinucleated, and small cells. In addition, frequent xanthic changes in the cytoplasm of the tumor cells, and widespread reticulin deposits and lymphocytic infiltrates in the stroma were characteristic features. Large areas of necrosis were also evident. However, mitotic figures were rare (1-2 mitoses per 10 high-power fields). Many tumor cells were positive for GFAP, and a number were positive for neurofilament protein and synaptophysin, indicating their neuronal differentiation. In addition, occasional tumor cells were positive for CD34. p53 protein was entirely negative in the tumor cells. In diagnosing this tumor histopathologically, differentiation between PXA and giant cell glioblastoma (GCG), a rare variant of glioblastoma, was problematic. However, considering the overall histopathological picture, a final diagnosis of PXA with anaplastic features was made. The present case indicates that PXA can occur as an intraventricular tumor, and suggests that in some instances, it would be very difficult to differentiate PXA and GCG histopathologically.

摘要

多形性黄色星形细胞瘤(PXA)是一种罕见的星形细胞瘤,通常发生在儿童和年轻成年人的大脑半球浅表部位,预后相对较好。我们报告了一例 52 岁男性患者的幕上、脑室肿瘤的不典型病例。肿瘤由多形性细胞组成,包括巨细胞,大多数为多核,还有小细胞。此外,肿瘤细胞的细胞质常有黄色变,基质中广泛存在网状纤维沉积和淋巴细胞浸润,也是其特征性表现。还可见大片状坏死。然而,有丝分裂象罕见(每 10 个高倍视野中有 1-2 个有丝分裂象)。许多肿瘤细胞呈 GFAP 阳性,部分呈神经丝蛋白和突触素阳性,提示其神经元分化。此外,偶尔肿瘤细胞呈 CD34 阳性。肿瘤细胞中 p53 蛋白完全阴性。在组织病理学诊断该肿瘤时,PXA 与胶质母细胞瘤(罕见的胶质母细胞瘤变异型)之间的鉴别存在问题。然而,考虑到整体组织病理学表现,最终诊断为具有间变特征的 PXA。本病例表明 PXA 可发生于脑室,提示在某些情况下,PXA 和 GCG 的组织病理学鉴别非常困难。

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