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软骨黏液样纤维瘤的细胞生物学与基质生物化学

Cell biology and matrix biochemistry of chondromyxoid fibroma.

作者信息

Söder S, Inwards C, Müller S, Kirchner T, Aigner T

机构信息

Department of Pathology, University of Erlangen-Nürnberg, Krankenhausstr. 8-10, D-91054 Erlangen, Germany.

出版信息

Am J Clin Pathol. 2001 Aug;116(2):271-7. doi: 10.1309/5BKG-E3CH-GGVA-1HDE.

Abstract

We studied matrix composition and gene expression pattern in chondromyxoid fibromas on the protein and the messenger RNA levels. We could clearly identify focal chondrocytic differentiation within chondromyxoid fibroma by the expression and deposition of type II collagen, which is a marker of chondrocytic cell differentiation. We also were able to show expression of collagen types I, III, and VI in the neoplasm. The major tumor portion was, however, characterized by the presence of hydrated proteoglycans and only minor amounts of collagens, a matrix composition responsible for the myxoid matrix appearance of most parts of these neoplasms. By analyzing cytoprotein expression, we found S-100 protein restricted to cells of the chondroid areas, suggesting S-100 protein staining to be of little help as a positive diagnostic marker for chondromyxoid fibroma. Our data show a specific matrix composition of chondromyxoid fibroma, not previously found in other mesenchymal neoplasms, including chondroblastoma, osteochondroma, enchondroma, and chondrosarcoma. This justifies chondromyxoid fibroma as a specific neoplastic entity, both clinically and biologically.

摘要

我们在蛋白质和信使核糖核酸水平上研究了软骨黏液样纤维瘤中的基质组成和基因表达模式。通过Ⅱ型胶原蛋白的表达和沉积,我们能够在软骨黏液样纤维瘤中清晰地识别出局灶性软骨细胞分化,Ⅱ型胶原蛋白是软骨细胞分化的标志物。我们还能够显示该肿瘤中Ⅰ型、Ⅲ型和Ⅵ型胶原蛋白的表达。然而,肿瘤的主要部分以富含水分的蛋白聚糖的存在为特征,仅含有少量胶原蛋白,这种基质组成导致了这些肿瘤大部分区域呈现黏液样基质外观。通过分析细胞蛋白表达,我们发现S-100蛋白仅限于软骨样区域的细胞,这表明S-100蛋白染色作为软骨黏液样纤维瘤的阳性诊断标志物作用不大。我们的数据显示了软骨黏液样纤维瘤特定的基质组成,这在包括软骨母细胞瘤、骨软骨瘤、内生软骨瘤和软骨肉瘤在内的其他间叶性肿瘤中未曾发现。这从临床和生物学角度都证明了软骨黏液样纤维瘤是一种特定的肿瘤实体。

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