Therapeutic Strategies Targeting Oncogenic Fusion Proteins in Sarcomas

Several drugs developed against cancer-specific molecular targets have been shown to offer survival benefits alone or in combination with standard treatments, especially for those cases where tumor pathogenesis is dominated by a single molecular abnormality. Such cases are also found in pediatric sarcomas which are characterized by specific chromosomal translocations generating oncogenic fusion proteins. In the majority of cases, these fusions are composed of transcription factors which can not be directly targeted by small-molecule compounds. However, the ability of transcription factors to regulate specific ta…