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Int J Clin Exp Pathol 2010;3(3):338-347
Review Article
Ewing sarcoma, an enigmatic malignancy of likely progenitor cell origin, driven by
transcription factor oncogenic fusions
Paul Jedlicka
Department of Pathology, University of Colorado Denver, Aurora CO 80045, USA.
Received March 5, 2009; accepted March, 2010; available online March, 2010
Abstract: Since its first description by James Ewing in 1921, Ewing Sarcoma has been a cryptic malignancy. A poorly differentiated tumor of
uncertain histogenesis and aggressive biologic behavior, it is the second most common malignancy of bone and soft tissue affecting
adolescents and young adults. Some two decades ago, the understanding of Ewing Sarcoma biology took a leap forward with the identification
of recurrent EWS/Ets fusions, which drive oncogenesis in this disease. A further leap forward occurred over the last half decade with the
application of gene silencing, global expression profiling and primary cell culture technologies to the study of this disease. Resulting work has
revealed EWS/Ets fusions to be surprisingly versatile regulators of gene expression, and has narrowed the search for the elusive cell of origin.
Improved understanding of EWS/Ets biology and relevant oncogenic pathways has in turn led to the development of targeted therapies,
including, recently, small molecules targeting key complexes involving the oncogenic fusion itself. In many respects still remaining an enigma,
Ewing Sarcoma is an important model for cancers originating in or manifesting progenitor-type cell features, and containing recurrent
oncogenic fusions, the latter a surprisingly expanding number. (IJCEP1003002).
Keywords: Ewing Sarcoma, fusion oncogene, transcription factor, progenitor cell
Full text PDF
Address all correspondence to:
Paul Jedlicka MD PhD
Department of Pathology
University of Colorado
Anschutz Medical Campus, PO Box 6511, MS 8104
Aurora CO 80045, USA.
Tel: 303-724-8161, Fax: 303-724-3712
E-mail: paul.jedlicka@ucdenver.edu
Review Article
Ewing sarcoma, an enigmatic malignancy of likely progenitor cell origin, driven by
transcription factor oncogenic fusions
Paul Jedlicka
Department of Pathology, University of Colorado Denver, Aurora CO 80045, USA.
Received March 5, 2009; accepted March, 2010; available online March, 2010
Abstract: Since its first description by James Ewing in 1921, Ewing Sarcoma has been a cryptic malignancy. A poorly differentiated tumor of
uncertain histogenesis and aggressive biologic behavior, it is the second most common malignancy of bone and soft tissue affecting
adolescents and young adults. Some two decades ago, the understanding of Ewing Sarcoma biology took a leap forward with the identification
of recurrent EWS/Ets fusions, which drive oncogenesis in this disease. A further leap forward occurred over the last half decade with the
application of gene silencing, global expression profiling and primary cell culture technologies to the study of this disease. Resulting work has
revealed EWS/Ets fusions to be surprisingly versatile regulators of gene expression, and has narrowed the search for the elusive cell of origin.
Improved understanding of EWS/Ets biology and relevant oncogenic pathways has in turn led to the development of targeted therapies,
including, recently, small molecules targeting key complexes involving the oncogenic fusion itself. In many respects still remaining an enigma,
Ewing Sarcoma is an important model for cancers originating in or manifesting progenitor-type cell features, and containing recurrent
oncogenic fusions, the latter a surprisingly expanding number. (IJCEP1003002).
Keywords: Ewing Sarcoma, fusion oncogene, transcription factor, progenitor cell
Full text PDF
Address all correspondence to:
Paul Jedlicka MD PhD
Department of Pathology
University of Colorado
Anschutz Medical Campus, PO Box 6511, MS 8104
Aurora CO 80045, USA.
Tel: 303-724-8161, Fax: 303-724-3712
E-mail: paul.jedlicka@ucdenver.edu
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