Pediatric sarcomas: translating molecular pathogenesis of disease to novel therapeutic possibilities.

ABSTRACT: Pediatric sarcomas represent a diverse group of rare bone and soft tissue malignancies. Although the molecular mechanisms that propel the development of these cancers are not well understood, identification of tumor-specific translocations in many sarcomas has provided significant insight into their tumorigenesis. Each fusion protein resulting from these chromosomal translocations is thought to act as a driving force in the tumor, either as an aberrant transcription factor (TF), constitutively active growth factor, or ligand-independent receptor tyrosine kinase. Identification of transcriptional targets or signaling pathways modulated by these oncogenic fusions has led to the discovery of potential therapeutic targets. Some of these targets have shown considerable promise in preclinical mo

SUBMITTER: Anderson JL 

PROVIDER: S-EPMC4283808 | biostudies-literature | 2012 Aug

REPOSITORIES: biostudies-literature