Ewing sarcoma: biology-based therapeutic perspectives.
Ewing sarcoma, a rare malignancy of childhood and adolescence, has attracted wide research interest. Tumor-specific chromosomal translocations generate aberrant EWS-ETS transcription factors, which alter intracellular signaling networks through gene and protein expression and are considered to be the primary tumor-initiating event. Ewing sarcoma therefore offers insights into principle molecular mechanisms of cancer development and maintenance. Still, despite long-standing research, biology-based targeted treatment strategies for Ewing sarcoma are only beginning to emerge. This article provides an overview of the biological basis and putative targeted treatment options.
Pediatric Hematology and Oncology, University Children's Hospital Münster, Münster, Germany.
Jenny
J
Potratz
Heribert
H
Jürgens
Alan
A
Craft
Uta
U
Dirksen
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000378:metabolism; D050783:Proto-Oncogene Proteins c-ets / Q000235:genetics / Q000378:metabolism; D034802:RNA-Binding Protein EWS / Q000235:genetics / Q000378:metabolism; D012512:Sarcoma, Ewing / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D014178:Translocation, Genetic / Q000235:genetics
D015514:Oncogene Proteins, Fusion; D050783:Proto-Oncogene Proteins c-ets; D034802:RNA-Binding Protein EWS