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孤立性纤维性肿瘤的超微结构谱:一种具有不同分化途径的独特血管周围肿瘤。

Ultrastructural spectrum of solitary fibrous tumor: a unique perivascular tumor with alternative lines of differentiation.

作者信息

Ide Fumio, Obara Kumi, Mishima Kenji, Saito Ichiro, Kusama Kaoru

机构信息

Department of Pathology, Tsurumi University School of Dental Medicine, 2-1-3 Tsurumi, Tsurumi-ku, Yokohama 230-8501, Japan.

出版信息

Virchows Arch. 2005 Jun;446(6):646-52. doi: 10.1007/s00428-005-1261-z. Epub 2005 May 21.

Abstract

Eight tumors diagnosed as solitary fibrous tumor (SFT) of the oral cavity were studied. Histologic spectrum was entirely comparable with the extrapleural SFT of other sites. One tumor had glomus tumor-like foci. Immunohistochemical results confirmed most of the previous observations, indicating characteristic expression of vimentin, CD34, bcl-2, and CD99. Factor XIIIa and alpha-smooth muscle actin were less commonly reactive and a very few cells were faintly positive for factor VIII-related antigen and Ulex europaeus agglutinin 1. All were essentially negative for S-100 protein, desmin, CD31, and CD68. In stark contrast to the conclusive immunoprofile, ultrastructural investigation of six tumors demonstrated considerable cellular heterogeneity. Other than fibroblasts, perivascular undifferentiated cells and pericytes predominated, but endothelial cells were regularly present. There was a distinctive proliferation of pericytic cells in four tumors, one of which had glomoid foci of myopericytes. The extreme increase in number of Weibel-Palade bodies occurred in voluminous capillary endothelium. Occasional single and clustered cells with consistent features of endothelium showed intracytoplasmic lumen formation. Such composite cells constituted an integral segment of richly vascularized SFT. Myofibroblastic form smooth muscle differentiation was present in only a minority of cells. From phenotypic analysis by electron microscopy, SFT may originate from a unique, perivascular multipotent mesenchyme sharing with its lineage with pericytes, fibroblasts, and infrequently, endothelium. Consequently, morphological features of SFT may become diversely varied by whether predominantly constituent cells are undifferentiated, pericytic or fibroblastic in nature.

摘要

对8例诊断为口腔孤立性纤维瘤(SFT)的肿瘤进行了研究。组织学谱与其他部位的胸膜外SFT完全可比。1例肿瘤有血管球瘤样灶。免疫组织化学结果证实了之前的大多数观察结果,显示波形蛋白、CD34、bcl-2和CD99有特征性表达。因子ⅩⅢa和α-平滑肌肌动蛋白较少有反应性,极少数细胞对因子Ⅷ相关抗原和荆豆凝集素1呈弱阳性。所有病例S-100蛋白、结蛋白、CD31和CD68基本为阴性。与明确的免疫表型形成鲜明对比的是,对6例肿瘤的超微结构研究显示出相当大的细胞异质性。除了成纤维细胞外,血管周围未分化细胞和周细胞占主导,但内皮细胞也经常出现。4例肿瘤中有明显的周细胞增殖,其中1例有肌周细胞的血管球样灶。大量毛细血管内皮细胞中出现了数量极多的Weibel-Palade小体。偶尔可见具有一致内皮细胞特征的单个和成簇细胞形成胞质内管腔。这种复合细胞构成了血管丰富的SFT的一个组成部分。肌成纤维细胞样平滑肌分化仅存在于少数细胞中。通过电子显微镜进行的表型分析表明,SFT可能起源于一种独特的血管周围多能间充质,其与周细胞、成纤维细胞以及偶尔与内皮细胞有共同的谱系。因此,根据主要组成细胞在本质上是未分化的、周细胞性的还是成纤维细胞性的,SFT的形态特征可能会有很大差异。

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