Abstract
While classically bone tumors were classified according to the differentiation and cellular morphology at the light microscopical level, it has become clear that over the past decade the array of knowledge which became available on the cytogenetic and molecular genetic level should influence tumor classification. This lead to an integrated approach in drafting the 2002 WHO nomenclature for bone tumors in which morphology was combined with the evolving genetic information. With regard to bone tumorigenesis distinct patterns can be recognized with regard to underlying genetics:
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balanced translocations such as in Ewing sarcoma, or aneurysmal bone cysts
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numerical chromosomal abnormalities like in adamantinoma
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complex karyotypes with as yet no identified starting point from a genetic point of view such as in osteosarcomas or central chondrosarcomas
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single gene events such as in fibrous dysplasia or osteochondroma
These different pathways in oncogenesis open an array of possibilities for studying the drivers in oncogenesis. Especially those pathways essential in keeping the proliferative state as opposed to the ones governing differentiation might proof to be potential targets for tailored drug treatment. Here high throughput screens focusing on kinase activity appear especially of interest. Within the EuroBoNeT consortium (www.eurobonet.eu) several working groups act transnationally together in unraveling these events. Important key elements of study are the molecular-genetic networks involved in skeletogenesis as well, which amongst others are studied within the consortium using in vitro systems as well as in vivo models using xenografted mouse models, mesenchymal stem cell models and models exploring zebrafish development.
Correspondence should be addressed to Professor Stefan Bielack, Olgahospital, Klinikum Stuttgart, Bismarkstrasse 8, D-70176 Stuttgart, Germany. Email: s.bielack@klinikum_stuttgart.de
