Expression of platelet-derived growth factor-alpha receptor in human osteosarcoma is not a predictor of outcome. The aims of this study were to examine the prognostic relevance of platelet-derived growth factor-α receptor (PDGFRA) expression in human osteosarcomas and to evaluate the mutation status of exon 12 and exon 18 of the PDGFRA gene.PDGFRA expression was examined in 100 human osteosarcomas by immunohistochemistry using paraffin embedded tumour tissues, and capillary sequencing of genomic DNA was performed to search for mutations in exons 12 and 18 of the PDGFRA gene.Ninety-six osteosarcomas showed PDGFRA expression ranging from 4% to 90% (mean 40%, median 37.5%, SD 27.11%). Furthermore, DNA sequence of exon 12 and exon 18 of the PDGFRA gene were not altered in 40 tumours with high PDGFRA expression. Overall and disease-free survival analysis did not reveal any differences between osteosarcoma patients with high PDGFRA expression and patients with low PDGFRA expression.The protein expression is not linked to mutations in exon 12 or exon 18 of PDGFRA gene. Therefore, treatment modalities based on the suppression of PDGFRA tyrosine kinase activity may need further investigation. PDGFRA expression is not a prognostic marker for osteosarcoma patients.