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[作为软骨形成性肿瘤生物学指标的基质基因表达模式]

[Matrix gene expression pattern as indicators of the biology of cartilage-forming tumors].

作者信息

Aigner T, Unni C C, Bjornsson J, Kirchner T

机构信息

Pathologisches Institut, Universität Erlangen.

出版信息

Verh Dtsch Ges Pathol. 1998;82:178-83.

Abstract

Mesenchymal cells can be characterized by the expression of specific sets of extracellular matrix (ECM) proteins. The aim of our study was to identify such expression pattern in cartilage-forming tumours in order to elucidate the biology of these neoplasms. In situ hybridisation and immunohistochemistry for various ECM components were performed on enchondromas, conventional chondrosarcomas of various grades and dedifferentiated chondrosarcomas. Chondroid areas of all investigated neoplasms showed strong expression of cartilage collagen types II and IX and aggrecan core and link protein. Focally, also the expression of other collagen subtypes such as collagen type X and collagen types I and III was observed. Non-chondroid areas of dedifferentiated chondrosarcomas lacked any expression of the cartilage-typical gene products and were instead positive for interstitial collagen types I, III, and VI. The hallmark of all investigated cartilaginous neoplasms was the expression of the chondrocyte-typical genes. Neoplastic chondrocytes showed the differentiation facettes similar to their physiological counterparts resulting in the high heterogeneity, which is characteristic for chondrogenic neoplasms. A different biology is suggested for dedifferentiated chondrosarcomas, in which the "dedifferentiated" portion is proposed to have a separate (e.g. fibroblastic) mesenchymal origin.

摘要

间充质细胞可通过特定的细胞外基质(ECM)蛋白表达谱来表征。我们研究的目的是确定软骨形成性肿瘤中的这种表达模式,以阐明这些肿瘤的生物学特性。对内生软骨瘤、不同分级的传统型软骨肉瘤和去分化软骨肉瘤进行了多种ECM成分的原位杂交和免疫组化检测。所有研究肿瘤的软骨样区域均显示出软骨Ⅱ型和Ⅸ型胶原以及聚集蛋白聚糖核心蛋白和连接蛋白的强表达。局部还观察到其他胶原亚型如Ⅹ型胶原以及Ⅰ型和Ⅲ型胶原的表达。去分化软骨肉瘤的非软骨样区域缺乏软骨典型基因产物的任何表达,而是Ⅰ型、Ⅲ型和Ⅵ型间质胶原呈阳性。所有研究的软骨性肿瘤的标志是软骨细胞典型基因的表达。肿瘤性软骨细胞表现出与其生理对应物相似的分化特征,导致高度异质性,这是软骨源性肿瘤的特征。去分化软骨肉瘤提示具有不同的生物学特性,其中“去分化”部分被认为具有独立的(如成纤维细胞样)间充质起源。

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