Ewing’s Sarcoma Family of Tumors: Molecular Targets Need Arrows

  • Jeffrey A. Toretsky
  • Aykut Üren


Ewing’s Sarcoma Family of Tumors (ESFT) are highly malignant tumors of bone and soft tissue that occur in children, adolescents, and young adults (Arndt and Crist 1999; Toretsky 2003). Currently, the standard therapy for ESFT patients is a five-drug regimen that consists of alternating cycles of doxorubicin/vincristine/cyclophosphamide and etoposide/ifosfamide over the course of approximately 9 months. Side effects include nausea, vomiting, and severe hematologic cytopenias, and patients often develop life-threatening infections while receiving chemotherapy. Patients who present with localized ESFT have approximately 70% disease-free survival. Patients who present with metastatic ESFT have a poor prognosis, reporting only 20% disease-free survival despite receiving intensive therapy (Grier et al. 2003). These clinical response rates have persisted for the past decade, even after patients received dose-intensifying chemotherapy and bone-marrow transplantation. Current treatment-related morbidity includes cardiac, musculoskeletal, and second malignancies (Fuchs et al. 2003). We need to discover novel therapeutic approaches to reduce treatment-related morbidity as well as improve overall survival. Novel therapies should exploit tumor vulnerability based on ESFT ontogeny, oncogenesis, and tumor-maintenance pathways.


Trail Receptor Clear Cell Sarcoma Desmoplastic Small Round Cell Tumor Uridine Phosphorylase Proteosome Inhibitor 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



The authors would like to thank Audrey Kubetin for her skillful assistance with this manuscript.


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© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Departments of Oncology and Pediatrics, Lombardi Comprehensive Cancer CenterGeorgetown UniversityWashingtonUSA

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