Aigner T, Loos S, Inwards C, Perris R, Perissinotto D, Unni K K, Kirchner T
Institute of Pathology, University of Erlangen-Nürnberg, Krankenhausstrasse 8-10, D-91054 Erlangen, Germany.
J Pathol. 1999 Dec;189(4):463-9. doi: 10.1002/(SICI)1096-9896(199912)189:4<463::AID-PATH476>3.0.CO;2-N.
Chondroblastoma is defined as a 'benign tumour, characterized by highly cellular and relatively undifferentiated tissue composed of rounded or polygonal chondroblast-like cells' and the 'presence of cartilaginous intercellular matrix' (WHO). An extensive analysis of the extracellular matrix composition and gene expression pattern of a large series of chondroblastoma cases shows, however, that type II collagen, which is the main component of any cartilage matrix, is not expressed by the neoplastic cells of this tumour entity and is not deposited into the extracellular tumour matrix. Instead, osteoid and fibrous matrix is formed, with its typical biochemical composition. The multifocal expression of aggrecan proteoglycan in most chondroblastomas explains the bluish, pseudo-chondroid appearance of some of the matrix-rich areas of chondroblastomas. This study did not show chondroid matrix formation or chondroblastic cell differentiation in chondroblastomas, suggesting that chondroblastoma should be classified as a specific bone-forming, rather than cartilage-forming neoplasm.
软骨母细胞瘤被定义为“一种良性肿瘤，其特征是由圆形或多边形软骨母细胞样细胞组成的高度细胞化且相对未分化的组织”以及“存在软骨细胞间基质”（世界卫生组织）。然而，对大量软骨母细胞瘤病例的细胞外基质组成和基因表达模式进行的广泛分析表明，作为任何软骨基质主要成分的II型胶原蛋白，在该肿瘤实体的肿瘤细胞中不表达，也不会沉积到肿瘤细胞外基质中。相反，会形成具有典型生化组成的类骨质和纤维基质。大多数软骨母细胞瘤中聚集蛋白聚糖蛋白多糖的多灶性表达解释了软骨母细胞瘤一些富含基质区域的蓝色、假软骨样外观。这项研究未显示软骨母细胞瘤中有软骨样基质形成或软骨母细胞分化，这表明软骨母细胞瘤应归类为一种特定的骨形成肿瘤，而非软骨形成肿瘤。
