Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2012:7:145-59.
doi: 10.1146/annurev-pathol-011110-130237. Epub 2011 Sep 19.

Molecular pathogenesis of Ewing sarcoma: new therapeutic and transcriptional targets

Stephen L Lessnick  1 , Marc Ladanyi
Affiliations
Review

Molecular pathogenesis of Ewing sarcoma: new therapeutic and transcriptional targets

Stephen L Lessnick et al. Annu Rev Pathol. 2012.

Abstract

Approximately one-third of sarcomas contain specific translocations. Ewing sarcoma is the prototypical member of this group of sarcomas; it was the first to be recognized pathologically as a singular entity and to have its signature translocation defined cytogenetically, which led to the identification of its key driver alteration, the EWS-FLI1 gene fusion that encodes this aberrant, chimeric transcription factor. We review recent progress in selected areas of Ewing sarcoma research, including the application of genome-wide chromatin immunoprecipitation analyses, to provide a comprehensive view of the EWS-FLI1 target gene repertoire, the identification of EWS-FLI1 target genes that may also point to therapeutically targetable pathways, and data from model systems as they relate to the elusive cell of origin of Ewing sarcoma and its possible similarities to mesenchymal stem cells.

PubMed Disclaimer

References

    1. Aman P, Panagopoulos I, Lassen C, Fioretos T, Mencinger M, et al. Expression patterns of the human sarcoma-associated genes FUS and EWS and the genomic structure of FUS. Genomics. 1996;37:1–-8. - PubMed
    1. Antonescu CR, Zhang L, Chang NE, Pawel BR, Travis W, et al. EWSR1-POU5F1 fusion in soft tissue myoepithelial tumors. A molecular analysis of sixty-six cases, including soft tissue, bone, and visceral lesions, showing common involvement of the EWSR1 gene. Genes Chromosomes Cancer. 2010;49:1114–-24. - PMC - PubMed
    1. Arvand A, Bastians H, Welford SM, Thompson AD, Ruderman JV, Denny CT. EWS/FLI1 up regulates mE2-C, a cyclin-selective ubiquitin conjugating enzyme involved in cyclin B destruction. Oncogene. 1998;17:2039–-45. - PubMed
    1. Arvand A, Denny CT. Biology of EWS/ETS fusions in Ewing's family tumors. Oncogene. 2001;20:5747–-54. - PubMed
    1. Aurias A. Chromosomal translocation in Ewing's sarcoma. N. Engl. J. Med. 1983;309:496–-97. - PubMed

Publication types

MeSH terms