Ewing’s Sarcoma Family of Tumors: Targeting Molecular Pathways and the Race for a Cure

Ewing’s sarcoma family of tumors (ESFT) is a malignant tumor of bone and soft tissue in children and adolescents that is characterized by molecular alterations that most commonly involve the EWS gene on chromosome 22. It is an aggressive tumor, with metastases present at diagnosis in 20–25% of cases. Modern treatment regimens for localized disease have resulted in significant improvement in survival. However, the pre­sence of metastasis is associated with morta­lity in 70–80% of patients. New insights into the pathogenesis and prolife­rative mechanisms of human cancers have led to the identification of a number of proteins acting as messenger molecules and modulators of tumor growth. Targeting these molecules in ESFT has the potential effect of controlling tumor growth. This chapter summarizes the current research on molecular pathogenesis, proliferation pathways and new investigational pharmacologic agents that are under development in the race for the cure of this malignancy.