Desmoplastic small round cell tumor(DSRCT)is an aggressive cancer that predominantly affects adolescents and young adults,typically developing at sites lined by mesothelium[1,2].DSRCT is genetically defined by a chrom...Desmoplastic small round cell tumor(DSRCT)is an aggressive cancer that predominantly affects adolescents and young adults,typically developing at sites lined by mesothelium[1,2].DSRCT is genetically defined by a chromosomal translocation that fuses the N-terminus of EWS RNA binding protein 1(EWSR1)to the C-terminus of Wilms tumor protein(WT1),forming EWSR1::WT1[3].This fusion encodes a potent transcription factor and is the only known driver of oncogenic transformation in DSRCT[4].The lack of a comprehensive understanding of DSRCT biology parallels its dismal survival rate(5%-20%)[1].These challenges are exacerbated by the absence of clinical trials,the limited systematic collection and analysis of DSRCT biomaterial[1],and the notable lack of specific diagnostic markers,necessitating resource-intensive molecular testing for an accurate diagnosis.展开更多
基金supported by grants from the Matthias-Lackas Foundation,the Dr.Leopold und Carmen Ellinger Foundation,the European Research Council(ERC CoG 2023#101122595)the Deutsche Forschungsgemeinschaft(DFG 458891500)+8 种基金the German Cancer Aid(DKH-70112257,DKH-7011411,DKH-70114278,DKH-70115315)the Dr.Rolf M.Schwiete foundation,the SMARCB1 association,the Ministry of Education and Research(BMBFSMART-CARE and HEROES-AYA)the Barbara and Wilfried Mohr foundation.The research team of Florencia Cidre-Aranaz was supported by the German Cancer Aid(DHK-70114111)the Dr.Rolf M.Schwiete Stiftung(2020-028 and 2022-31)supported by the Cancer Grand Challenges partnership funded by Cancer Research UK,the National Cancer Institute,the Scientific Foundation of the Spanish Association Against Cancer And KiKa(Children Cancer Free Foundation)Florian Henning Geyer,Tobias Faehling,Endrit Vinca,and Alina Ritter were supported by the German Academic Scholarship Foundation.In addition,Endrit Vinca was supported by scholarships from the Heinrich F.C.Behr foundation and the Rudolf and Brigitte Zenner foundation,Tobias Faehling by the Heinrich F.C.Behr foundationFlorian Henning Geyer and Alina Ritter are supported by the German Cancer Aid through the‘Mildred-Scheel-Doctoral Program’(DKH-70114866)This project is co-funded by the European Union(ERC,CANCER-HARAKIRI,101122595)。
文摘Desmoplastic small round cell tumor(DSRCT)is an aggressive cancer that predominantly affects adolescents and young adults,typically developing at sites lined by mesothelium[1,2].DSRCT is genetically defined by a chromosomal translocation that fuses the N-terminus of EWS RNA binding protein 1(EWSR1)to the C-terminus of Wilms tumor protein(WT1),forming EWSR1::WT1[3].This fusion encodes a potent transcription factor and is the only known driver of oncogenic transformation in DSRCT[4].The lack of a comprehensive understanding of DSRCT biology parallels its dismal survival rate(5%-20%)[1].These challenges are exacerbated by the absence of clinical trials,the limited systematic collection and analysis of DSRCT biomaterial[1],and the notable lack of specific diagnostic markers,necessitating resource-intensive molecular testing for an accurate diagnosis.
