title: Molecular pathogenesis of osteosarcoma.
abstract: Osteosarcoma is a devastating but rare disease, whose study has illuminated both the basic biology and clinical management of cancer over the past 30 years. These contributions have included insight into the roles of key cancer genes such as the retinoblastoma tumor suppressor gene and TP53, the identification of familial cancer syndromes implicating DNA helicases, and dramatic improvements in survival by the use of adjuvant chemotherapy. This review provides a synoptic overview of our current understanding of the molecular causes of osteosarcoma, and suggests future directions for study.
authors.affiliation: Ian Potter Foundation Centre for Cancer Genomics and Predictive Medicine and Research Division, Peter MacCallum Cancer Centre, Melbourne, Australia.
authors.forename: Maya
authors.initials: M
authors.lastname: Kansara
authors.forename: David M
authors.initials: DM
authors.lastname: Thomas
mesh_terms: D000818:Animals; D001816:Bloom Syndrome / Q000235:genetics / Q000473:pathology; D001859:Bone Neoplasms / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D002869:Chromosome Aberrations; D003598:Cytoskeletal Proteins / Q000235:genetics / Q000378:metabolism; D020022:Genetic Predisposition to Disease; D006801:Humans; D020782:Matrix Metalloproteinases / Q000235:genetics / Q000378:metabolism; D051379:Mice; D009154:Mutation; D009361:Neoplasm Invasiveness; D009362:Neoplasm Metastasis; D025581:Neurofibromin 2 / Q000235:genetics / Q000378:metabolism; D010001:Osteitis Deformans / Q000235:genetics / Q000473:pathology; D012516:Osteosarcoma / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D053484:RecQ Helicases / Q000235:genetics / Q000378:metabolism; D016160:Retinoblastoma Protein / Q000235:genetics / Q000378:metabolism; D011038:Rothmund-Thomson Syndrome / Q000235:genetics / Q000473:pathology; D016159:Tumor Suppressor Protein p53 / Q000235:genetics / Q000378:metabolism; D014898:Werner Syndrome / Q000235:genetics / Q000473:pathology; D000099149:Ezrin
chemical_list: D003598:Cytoskeletal Proteins; D020782:Matrix Metalloproteinases; D025581:Neurofibromin 2; D053484:RecQ Helicases; D016160:Retinoblastoma Protein; D016159:Tumor Suppressor Protein p53; D000099149:Ezrin