Leiden University Scholarly Publications

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Salvatore, R. (2010)

Chondroblastoma and chondromyxoid fibroma: disentangling the neoplastic chondrogenesis of two rare cartilaginous tumours

Doctoral Thesis
The scope of this study was to disentangle neoplastic chondrogenesis in two rare cartilaginous tumours: chondroblastoma and chondromyxoid fibroma. It was addressed: 1 The spectrum of phenotypic differentiation in chondroblastoma and chondromyxoid fibroma, 2 The signalling pathways driving chondroblastoma and chondromyxoid fibroma pathogenesis, 3 The somatic genetic background underlying chondroblastoma and chondromyxoid fibroma. The findings of this study and the literature suggest that chondroblastoma and chondromyxoid fibroma may originate from a mesenchymal stem cell precursor. An as yet unknown genetic event may induce neoplastic transformation and: 1) on the one hand activate signalling cascade crucial for neoplastic cell survival (i.e. PTHLH, TGF-_, etc.) and commit cells towards cartilage (expression of cartilage-specific molecules), 2) on the other hand activate an expression profile of __non-cartilage specific__ molecules distinct for chondroblastoma and chondromyxoid...Show moreThe scope of this study was to disentangle neoplastic chondrogenesis in two rare cartilaginous tumours: chondroblastoma and chondromyxoid fibroma. It was addressed: 1 The spectrum of phenotypic differentiation in chondroblastoma and chondromyxoid fibroma, 2 The signalling pathways driving chondroblastoma and chondromyxoid fibroma pathogenesis, 3 The somatic genetic background underlying chondroblastoma and chondromyxoid fibroma. The findings of this study and the literature suggest that chondroblastoma and chondromyxoid fibroma may originate from a mesenchymal stem cell precursor. An as yet unknown genetic event may induce neoplastic transformation and: 1) on the one hand activate signalling cascade crucial for neoplastic cell survival (i.e. PTHLH, TGF-_, etc.) and commit cells towards cartilage (expression of cartilage-specific molecules), 2) on the other hand activate an expression profile of __non-cartilage specific__ molecules distinct for chondroblastoma and chondromyxoid fibroma, respectively . Subsequently __passenger-related__ structural changes are hypothesized to occur.Show less
Cartilaginous tumours
Chondroblastoma
Chondromyxoid fibroma
Neoplastic chondrogenesis
All authors
Salvatore, R.
Supervisor
Hogendoorn, P.C.W.
Qualification
Doctor (dr.)
Awarding Institution
Faculty of Science, Leiden University
Date
2010-06-22