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低度恶性肌纤维肉瘤中的纤维连接桥粒:一例报告

Fibronexus in low-grade myofibrosarcoma: a case report.

作者信息

Watanabe Kazuo, Tanaka Mizuko, Takashi Kusakabe, Yamada Hitoshi, Tajino Takahiro

机构信息

Pathology Division, Fukushima Medical University Hospital, oka, Fukushima City, Fukushima, Japan.

出版信息

Ultrastruct Pathol. 2008 May-Jun;32(3):97-100. doi: 10.1080/01913120701829301.

Abstract

Fibronexus (fibronexus junction) has been thought to be a characteristic ultrastructural feature of myofibroblasts, but it is controversial as to whether fibronexus is a characteristic of various myofibroblastic tumors. We report here a case of low-grade myofibrosarcoma with fibronexus arising in the right arm of an 80-year-old man. Histologically, the tumor was composed of relatively uniform and slender spindle cells arranged in fascicles. The nuclei with fusiform and tapered shapes were mildly hyperchromatic, but never exhibited pleomorphism. Mitotic figures were common, but no atypical mitosis was identified. At the tumor periphery, tumor cells had invaded into the surrounding skeletal muscle tissue. Tumor cells were positive diffusely for alpha-smooth muscle actin and less intensely for desmin, but were negative for h-caldesmon and S-100 protein. Ultrastructurally, tumor cells had well developed cytoplasmic organelles and varying amounts of peripheral or subplasmalemmal bundles of thin myofilaments with focal density. In addition, well formed, long fibronectin fibrils adjacent to the cell surface and fibronexus contacting intracellular myofilaments were easily identified. We believe that fibronexus is a useful ultrastructural feature for differentiating myofibrosarcoma from other myogenic sarcomas.

摘要

纤维连接(纤维连接点)一直被认为是肌成纤维细胞的一个特征性超微结构特征,但纤维连接是否为各种肌成纤维细胞性肿瘤的特征仍存在争议。我们在此报告一例发生于一名80岁男性右臂的伴有纤维连接的低级别肌纤维肉瘤病例。组织学上,肿瘤由排列成束状的相对均匀且细长的梭形细胞组成。呈梭形和锥形的细胞核轻度深染,但从未表现出多形性。有丝分裂象常见,但未发现非典型有丝分裂。在肿瘤周边,肿瘤细胞已侵入周围的骨骼肌组织。肿瘤细胞α平滑肌肌动蛋白弥漫性阳性,结蛋白阳性程度较低,但h-钙调蛋白和S-100蛋白阴性。超微结构上,肿瘤细胞有发育良好的细胞质细胞器以及不同数量的外周或近质膜的细肌丝束,伴有局灶性致密物质。此外,易于识别细胞表面相邻的结构良好的长纤连蛋白原纤维以及与细胞内肌丝相连的纤维连接。我们认为纤维连接是将肌纤维肉瘤与其他肌源性肉瘤区分开来的一个有用的超微结构特征。

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