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脑膜血管外皮细胞瘤的免疫表型谱:与纤维型脑膜瘤和脑膜孤立性纤维性肿瘤的比较。

The immunophenotypic spectrum of meningeal hemangiopericytoma: a comparison with fibrous meningioma and solitary fibrous tumor of meninges.

作者信息

Perry A, Scheithauer B W, Nascimento A G

机构信息

Department of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.

出版信息

Am J Surg Pathol. 1997 Nov;21(11):1354-60. doi: 10.1097/00000478-199711000-00010.

Abstract

Despite controversy regarding its histogenesis, meningeal hemangiopericytoma (HPC) is a well-defined clinicopathologic entity exhibiting high rates of recurrence and late extracranial metastasis. It must be distinguished from several benign neoplasms, particularly fibrous meningioma (FM) and solitary fibrous tumor (SFT). To determine the immunoprofile of HPC, we studied 27 meningeal examples, including 13 low-grade and 14 high-grade tumors. For comparison, 20 FMs and eight SFTs of the meninges were also evaluated. The immunotype of HPC included vimentin (85%), factor XIIIa (78%) in individual scattered cells, Leu-7 (70%), and CD34 (33%) in a weak, patchy pattern. Focal desmin and cytokeratin positivity was only occasionally encountered (20% each). The SFT shared a similar immunophenotype, except that CD34 expression (100%) was characteristically strong and diffuse. The FM characteristically expressed epithelial membrane antibody (EMA) (80%) and S-100 protein (80%); CD34 reactivity (60%) was patchy and weak. Both within and among all three tumor types, MIB-1 labeling indices varied widely. Specifically, they were unrelated to tumor grade in HPC. Significant reactivity for p53 protein was detected in 52% of HPCs, 17% of SFTs, and 5% of FMs. Meningeal HPC exhibits a distinct antigenic profile, one enabling the exclusion of other entities in nearly all cases. The rare expression of desmin or cytokeratin in HPC suggests either the occurrence of divergent differentiation or, less likely, the possibility that its distinctive morphology is but a phenotype shared by several types of meningeal sarcoma.

摘要

尽管关于其组织发生存在争议,但脑膜血管外皮细胞瘤(HPC)是一种明确的临床病理实体,具有高复发率和晚期颅外转移率。它必须与几种良性肿瘤相鉴别,特别是纤维性脑膜瘤(FM)和孤立性纤维性肿瘤(SFT)。为了确定HPC的免疫表型,我们研究了27例脑膜病例,包括13例低级别和14例高级别肿瘤。作为对照,还评估了20例脑膜FM和8例脑膜SFT。HPC的免疫类型包括波形蛋白(85%)、个别散在细胞中的因子ⅩⅢa(78%)、Leu-7(70%)以及呈弱片状模式的CD34(33%)。仅偶尔见到局灶性结蛋白和细胞角蛋白阳性(各20%)。SFT具有相似的免疫表型,只是CD34表达(100%)具有特征性的强阳性和弥漫性。FM特征性地表达上皮膜抗体(EMA)(80%)和S-100蛋白(80%);CD34反应性(60%)呈片状且较弱。在所有三种肿瘤类型内部及之间,MIB-1标记指数差异很大。具体而言,它们与HPC的肿瘤级别无关。在52%的HPC、17%的SFT和5%的FM中检测到p53蛋白的显著反应性。脑膜HPC表现出独特的抗原谱,几乎在所有病例中都能借此排除其他实体。HPC中结蛋白或细胞角蛋白的罕见表达提示要么发生了分化异常,要么可能性较小的是,其独特形态只是几种脑膜肉瘤共有的一种表型。

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