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PMID: 21942527 Published · ppublish English Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Review

Molecular pathogenesis of Ewing sarcoma: new therapeutic and transcriptional targets.

Annual review of pathology ·Vol. 7 ·2012-00-00 ·Pages 145-59

Lessnick SL, Ladanyi M

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.

MeSH Terms
Animals Bone Neoplasms/etiology,metabolism,pathology Chromatin Immunoprecipitation Gene Expression Regulation, Neoplastic Humans Mesenchymal Stem Cells/pathology Oncogene Proteins, Fusion/genetics,metabolism Proto-Oncogene Protein c-fli-1/genetics,metabolism RNA-Binding Protein EWS/genetics,metabolism Sarcoma, Ewing/etiology,metabolism,pathology Transcription Factors/genetics,metabolism Translocation, Genetic
Chemicals
EWS-FLI fusion protein Oncogene Proteins, Fusion Proto-Oncogene Protein c-fli-1 RNA-Binding Protein EWS Transcription Factors
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Lessnick Stephen L
Center for Children's Cancer Research at Huntsman Cancer Institute, Department of Oncological Sciences, University of Utah School of Medicine, Salt Lake City, Utah 84112, USA. [email protected]
Ladanyi Marc
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Article Info
Journal
Annual review of pathology
Abbr.
Annu Rev Pathol
ISSN
1553-4014
DOI
10.1146/annurev-pathol-011110-130237
Email
[email protected]
Published
2012-00-00
Epub
2011-00-19
Pages
145-59
Language
English
Region
United States
NLM ID
101275111
PMCID
PMC3555146
Subset
IM
Grants
NCI NIH HHS · P30-CA042014 · United States
NCI NIH HHS · R21 CA138295 · United States
NCI NIH HHS · R01CA140394 · United States
NCI NIH HHS · P01 CA106450 · United States
NCI NIH HHS · R01 CA140394 · United States
NCI NIH HHS · P30 CA042014 · United States
External Links
PubMed source DOI: 10.1146/annurev-pathol-011110-130237 PMC Full Text View journal details
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