Protocol Number: 03-C-0216 ![]()
It is clear that improved understanding of the basic biology of Ewing sarcoma will ultimately lead to improved treatment approaches to this disease. With the identification of the invariant EWS-ETS fusion gene in these tumors, the search for new downstream target genes is ongoing with hopes of identifying new molecular treatment targets for drug development. Many important questions remain to be resolved, including the effect of translocation type on prognosis, the effect of submicroscopic molecular-only disease on long-term outcome and the identification of molecular targets and molecular determinants of prognosis. Objectives: -To develop a mechanism to collect and distribute tumor specimens to various investigators and a system to prioritize and develop quality control measures for central data reporting of studies undertaken. -To determine the prognostic significance of translocation subtype in Ewing sarcoma. -To determine the prognostic significance of MRD detection in bone marrow specimens by RT-PCR -To determine if serum levels of IGF are of significance in the outcome of patients with Ewing sarcoma. -To determine if RNA expression profiles performed on diagnostic specimens will allow for the identification of newer prognostic categories and potentially new molecular targets for treatment in Ewing sarcoma. -To identify new targets for therapy. -To identify a bank of Ewing sarcoma xenografts in SCID mice. -To establish clinical proteomics as a resource for investigations of altered signaling molecules in the pathogenesis of Ewing sarcoma. Eligibility: -Newly diagnosed or recurrent Ewing sarcoma patients, prior to treatment. -Specimen requirements consisting of paraffin embedded block or 20 unstained slides and pretreatment blood and tissue specimens. -For patients who enroll on a Ewing sarcoma therapeutic trial : Agreement to obtain specimens at additional designated time-points during therapy, at the time of surgery, at the completion of therapy and at relapse. Design: This study is designed to analyze biological factors of Ewing tumors, and for those patients enrolled on the local and metastatic Ewing sarcoma protocols, to relate the tumor characteristics and treatment outcomes. With this information it will be possible to further refine the aims of the next biology study. This study will also establish a bank for Ewing sarcoma tumors.
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