期刊论文详细信息
Frontiers in Cell and Developmental Biology
Ewing sarcoma from molecular biology to the clinic
Cell and Developmental Biology
Bénédicte Brounais-Le Royer1  Benjamin Ory1  Anaïs Postec1  François Lamoureux1  Mathilde Mullard1  Laura Regnier1  Marc Baud’huin1  Steven Georges1  Françoise Rédini1  Maryne Dupuy2  Franck Verrecchia3 
[1] Nantes Université, Inserm UMR 1307, CNRS UMR 6075, CRCI2NA, Université d'Angers, Nantes, France;franck.verrecchia@univ-nantes.fr;null;
关键词: ewing sarcoma;    EWS-FLI1;    clinical;    molecular biology;    cellular biology;   
DOI  :  10.3389/fcell.2023.1248753
 received in 2023-06-27, accepted in 2023-08-30,  发布年份 2023
来源: Frontiers
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【 摘 要 】

In Europe, with an incidence of 7.5 cases per million, Ewing sarcoma (ES) is the second most common primary malignant bone tumor in children, adolescents and young adults, after osteosarcoma. Since the 1980s, conventional treatment has been based on the use of neoadjuvant and adjuvant chemotherapeutic agents combined with surgical resection of the tumor when possible. These treatments have increased the patient survival rate to 70% for localized forms, which drops drastically to less than 30% when patients are resistant to chemotherapy or when pulmonary metastases are present at diagnosis. However, the lack of improvement in these survival rates over the last decades points to the urgent need for new therapies. Genetically, ES is characterized by a chromosomal translocation between a member of the FET family and a member of the ETS family. In 85% of cases, the chromosomal translocation found is (11; 22) (q24; q12), between the EWS RNA-binding protein and the FLI1 transcription factor, leading to the EWS-FLI1 fusion protein. This chimeric protein acts as an oncogenic factor playing a crucial role in the development of ES. This review provides a non-exhaustive overview of ES from a clinical and biological point of view, describing its main clinical, cellular and molecular aspects.

【 授权许可】

Unknown   
Copyright © 2023 Dupuy, Lamoureux, Mullard, Postec, Regnier, Baud’huin, Georges, Brounais-Le Royer, Ory, Rédini and Verrecchia.

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