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软组织和骨黏液样软骨肉瘤:11例回顾性研究

Myxoid chondrosarcoma of soft tissue and bone: a retrospective study of 11 cases.

作者信息

Abramovici L C, Steiner G C, Bonar F

机构信息

Department of Pathology, Hospital for Joint Diseases, New York, NY 10003, USA.

出版信息

Hum Pathol. 1995 Nov;26(11):1215-20. doi: 10.1016/0046-8177(95)90196-5.

Abstract

This article describes 11 cases of myxoid chondrosarcoma (MCS), with 10 arising in soft tissues and one developing in bone. Most of the tumors (six) were located in the lower extremities. Two lesions developed in the fingers, a previously unreported location for MCS. Four cases showed secondary bone destruction, which is a rare feature of this tumor. S100 protein was expressed by tumor cells in all the specimens. Four out of eight tumors studied by electron microscopy contained intracisternal microtubular structures. Two tumors showed areas of spindle cell proliferation that merged with the areas of typical myxoid pattern. The cells in these areas had fibroblastic/myofibroblastic features by electron microscopy and were found to express cytokeratin by immunohistochemistry. The concomitant expression of cytokeratin and S100 protein in the spindle cells suggests that they represent a less differentiated cartilaginous component with unusual features. The clinical significance of the presence of such spindle cell areas presently remains unknown. Although myxoid chondrosarcoma is a slow-growing tumor, it has a high potential for metastases. Four of 11 patients in this series developed metastases.

摘要

本文描述了11例黏液样软骨肉瘤(MCS),其中10例发生于软组织,1例发生于骨。大多数肿瘤(6例)位于下肢。有2例病变发生在手指,这是MCS以前未报道过的发病部位。4例显示继发性骨破坏,这是该肿瘤的罕见特征。所有标本中的肿瘤细胞均表达S100蛋白。在8例经电子显微镜研究的肿瘤中,有4例含有池内微管结构。2例肿瘤显示梭形细胞增殖区域与典型黏液样模式区域融合。这些区域的细胞在电子显微镜下具有成纤维细胞/肌成纤维细胞特征,并且通过免疫组织化学发现它们表达细胞角蛋白。梭形细胞中细胞角蛋白和S100蛋白的同时表达表明它们代表具有异常特征的分化程度较低的软骨成分。目前,这种梭形细胞区域存在的临床意义尚不清楚。尽管黏液样软骨肉瘤是一种生长缓慢的肿瘤,但它具有很高的转移潜能。该系列11例患者中有4例发生了转移。

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