Predictors of overall survival in patients diagnosed with desmoplastic small round cell tumor (DSRCT).

10582 Background: DSRCT is a rare, aggressive, and poorly understood neoplasm characterized by the EWS-WT1 translocation. Prognosis is poor despite upfront multimodality therapy with surgery, radiation (RT), and high-dose chemotherapy. We performed a retrospective review of patients (pts) with DSRCT at Memorial Sloan Kettering Cancer Center (MSKCC) to assess the association between overall survival (OS) and various clinical and therapeutic variables. Methods: We collected demographic/clinical data on pts diagnosed with DSRCT from 1995-2011 who had pathologic review and ≥1 oncologic consultation at MSKCC. Living pts were censored at time of last contact. First-line therapies were classified as follows: optimal (R0/R1) vs suboptimal (R2) surgery; consolidative RT; auto transplant; and chemotherapy: high-dose cyclophosphamide (HDCy) vs. standard Ewing’s regimen [sER] vs other. Univariate (UVA) and multivariate (MVA) analyses were performed. Results: 191 patients were identified; 152 (80%) male, median age 22...