Abstract: Objective To investigate the clinical characteristics and prognostic factors affecting the survival of the desmoplastic small round cell tumor (DSRCT). Methods Clinical data of five patients with DSRCT who were diagnosed and treated in Fujian Tumor Hospital from January 1998 to February 2012 were collected．126 cases with complete clinical data were collected and reviewed from 90 published reports.Therefore,totally 131 patients with DSRCT were analysed．Kaplan-Meier method was applied to univariate analysis.Log rank was used to compare curves between these groups.Cox regression was applied to multivariate prognostic analysis.Statistical significance was considered at P<0.05. Results In the univariate analysis,tumor location,number of tumors,size of tumor,with or without metastases,and whether or not to accept radical surgery,whether or not to accept chemotherapy and radiation therapy are the prognostic factors.In the Cox proportional hazards regression model for multivariate analysis,whether or not to accept radical surgery relative risk (RR) 13.250,95% confidence interval (CI) 5.675-30.937,P=0.000,whether or not to undergo chemotherapy(RR=5.714,95% CI=3.165-10.317,P=0.000) and whether or not to undergo radiation therapy(RR=7.498,95% CI=2.628-21.389,P=0.000) are the prognostic factors in DSRCT. Conclusion Whether or not to accept radical surgery,chemotherapy and radiation therapy are the independent prognostic factors in DSRCT.Currently,early diagnosis,striving for radical surgery,systemic chemotherapy and radiation therapy is the reasonable way to prolong survival.