The project through collaborative studies will define prognostic markers and new therapeutic targets in the Ewing's sarcoma family of tumours (ESFT) to provide rigorous scientific justifications for the development of clinical trials for this rare disease, which is manifested for the most part in children. The main objective of this project is to evaluate the prognostic relevance of selected markers (EWS/FLI-1, secondary genetic alterations, CD99, IGF-IR, NOVH, erbB-2 and TTF1) and the effectiveness of therapeutic approaches targeting some of these molecules. The prognostic value of these markers will be evaluated in retrospective and prospective series of ESFT patients treated across the participating centres. Through statistical analysis, we will verify which factors have the highest prognostic impact in ESFT patients, in terms of overall survival, disease progression, and chemosensitivity. In order to provide the necessary rationale for the forthcoming application of new therapies, the preclinical effectiveness of new drugs (Herceptin) and strategies targeting molecules (CD99, IGF-IR, EWS/FLI1) found to be critical for ESFT will be evaluated. Another major goal of the project is the construction of ESFT c- DNA microarrays and tissue arrays, which will be used for the analysis of different hystological subtypes of ESFT, primary and metastatic tumors and poor and good responders to chemotherapy. Therefore, the expected results are: 1) identification of prognostic factors in ESFT; 2) definition of patient selection criteria 3) creation of new therapeutic bullets against ESFT; 4) identification of new therapies; 5) creation of new tools for the diagnosis and screening of high-risk groups. This will lead to: 1) the definition of forthcoming risk-adapted strategies and targeted molecular treatments to be advantageously combined with established therapies; 2) improved quality of life and survival for ESFT patients; 3) prevention on ris#'
Keywords:
Ewing tumour; prognosis; targeted therapies
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