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[原发性脑膜血管外皮细胞瘤的临床病理研究]

[Clinicopathological study of primary meningeal hemangiopericytoma].

作者信息

Shi Qun-li, Chen Xu-dong, Lu Zhen-feng, Meng Kui, Jin Xing-zao, Yan Xiao-wen, Zhou Xiao-jun, Sheng Chun-ning

机构信息

Department of Pathology, Nanjing Military General Hospital of PLA, School of Clinical Medicine, Nanjing University, Jiangsu, Nanjing, 210002, P. R. China.

出版信息

Ai Zheng. 2002 Oct;21(10):1116-9.

Abstract

BACKGROUND AND OBJECTIVES

Meningeal hemangiopericytoma is an uncommon tumor. This study was designed to investigate the clinicopathological and biology behavior of primary meningeal hemangiopericytoma.

METHODS

Clinical data, combined with histopathology and immunohistochemistry of 20 cases of meningeal hemangiopericytoma were reviewed, in which 4 specimens were examined with electron microscope.

RESULTS

The average age of patients with primary meningeal hemangiopericytoma was 42.4 year-old (21-69 years). The ratio of male to female was 1.2: 1. Most of the patients went to hospital for the symptoms of central nervous system such as headache. The tumor could occur on any locus of the cranial or spinal dura. Grossly, many of the tumors had capsule, whose testure were tenacious, and part of them looks like fish meat. Histopathologically, the small vascular spaces in the tumor were rich, the typical antler-liked vessel could be found, the short-spindle tumor cells were around the vessel, and distributed by radiation-shape. The tumor cells were pleomorphic and atypical, and could be found mitotic activity. Staining of argyrophilic fiber: the argyrophilic fiber surrounded single tumor cell, and distributed by radiation-shape around vessel. Immunohistochemistry showed negative for S-100 protein, F VIII, EMA, GFAP and CD34, while Vim was positive. Electron microscopically, the rich bundles of 10nm long intermediate filaments could be found in tumor cells. The exobasallamina, of cells were evidenced, and distributed around single cell. Follow-up, 8 of 17 cases were relapsed (47.1%).

CONCLUSION

Meningeal hemangiopericytoma is a low-malignant tumor original from meningeal mesenchymal tissue. The features of histopathology, immunphenotype and ultrastructure are similar to hemangiopericytoma of the soft tissue.

摘要

背景与目的

脑膜血管外皮细胞瘤是一种罕见肿瘤。本研究旨在探讨原发性脑膜血管外皮细胞瘤的临床病理及生物学行为。

方法

回顾20例脑膜血管外皮细胞瘤的临床资料,并结合组织病理学和免疫组化检查,其中4例标本进行了电镜检查。

结果

原发性脑膜血管外皮细胞瘤患者的平均年龄为42.4岁(21 - 69岁)。男女比例为1.2:1。大多数患者因头痛等中枢神经系统症状就诊。肿瘤可发生于颅或脊膜的任何部位。大体上,许多肿瘤有包膜,质地坚韧,部分呈鱼肉样。组织病理学上,肿瘤内小血管丰富,可见典型的鹿角样血管,短梭形肿瘤细胞围绕血管呈放射状分布。肿瘤细胞多形性且不典型,可见核分裂象。嗜银纤维染色:嗜银纤维围绕单个肿瘤细胞,并围绕血管呈放射状分布。免疫组化显示S - 100蛋白、F VIII、EMA、GFAP和CD34均为阴性,而波形蛋白为阳性。电镜下,肿瘤细胞内可见丰富的10nm长的中间丝束。细胞外基膜可见,围绕单个细胞分布。随访17例中有8例复发(47.1%)。

结论

脑膜血管外皮细胞瘤是一种起源于脑膜间叶组织的低恶性肿瘤。其组织病理学、免疫表型及超微结构特征与软组织血管外皮细胞瘤相似。

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