[Ewing's tumours, genetic and cellular aspects].
Ewing's tumour is the second most frequent primary tumour of bone. It is associated in 85% of cases with a specific and recurrent chromosome translocation, a t(11; 22)(q24; q12) which generates a fusion gene between the 5' part of EWS and the 3' part of FLI-1, a member of the ETS family. Less frequently, this gene fusion involves EWS and another member of the ETS family which can be: ERG, ETV1, E1AF or FEV depending on the cases. The EWS-ETS fusion is causative in the development of Ewing's tumour. Its mechanism of action mainly relies on the abnormal transcription regulation of key target genes which are involved in the regulation of cell cycle, signal transduction, migration. The cellular context within which EWS-FLI-1 exerts its oncogenic action is a long standing matter of debate. Recent data converge to suggest that the Ewing cell origin is a mesenchymal stem cell.
Delattre
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D001859:Bone Neoplasms / Q000235:genetics* / Q000473:pathology; D002471:Cell Transformation, Neoplastic / Q000235:genetics; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D059630:Mesenchymal Stem Cells / Q000473:pathology; D014411:Neoplastic Stem Cells / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000502:physiology*; D051789:Proto-Oncogene Protein c-fli-1 / Q000235:genetics / Q000502:physiology*; D034802:RNA-Binding Protein EWS / Q000235:genetics*; D012512:Sarcoma, Ewing / Q000235:genetics* / Q000473:pathology; D014157:Transcription Factors / Q000235:genetics / Q000502:physiology; D014178:Translocation, Genetic
C111598:EWS-ATF1 fusion protein, human; C450460:EWS-ERG fusion protein, human; C419546:EWS-FEV fusion protein, human; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D014157:Transcription Factors