Chondroblastoma is defined as a 'benign tumour, characterized by highly cel
lular and relatively undifferentiated tissue composed of rounded or polygon
al chondroblast-like cells' and the 'presence of cartilaginous intercellula
r matrix' (WHO). An extensive analysis of the extracellular matrix composit
ion and gene expression pattern of a large series of chondroblastoma cases
shows, however, that type II collagen, which is the main component of any c
artilage matrix, is not expressed by the neoplastic cells of this tumour en
tity and is not deposited into the extracellular tumour matrix. Instead, os
teoid and fibrous matrix is formed, with its typical biochemical compositio
n. The multifocal expression of aggrecan proteoglycan in most chondroblasto
mas explains the bluish, pseudo-chondroid appearance of some of the matrix-
rich areas of chondroblastomas, This study did not show chondroid matrix fo
rmation or chondroblastic cell differentiation in chondroblastomas, suggest
ing that chondroblastoma should be classified as a specific bone-forming, r
ather than cartilage-forming neoplasm, Copyright (C) 1999 John Wiley: & Son
s, Ltd.