Multidisciplinary diagnosis and combined treatment of desmoplastic small round-cell tumor

Objective：To summarize the experience of diagnosis and treatment of desmoplastic small round-cell tumor (DSRCT) in our single clinical center. Methods：The clinical data and survival time of patients with pathologically confirmed DSRCT admitted to the Department of Oncology, Zhongshan Hospital, Fudan University between January 2010 and December 2018 were analyzed. A total of 11 patients with DSRCT were selected, including 7 males (64%) and 4 females (36%). The male/female ratio of DSRCT was 1.75∶1. The median age of onset of patients was 27 (12-49) years. the end of study, there were 4 survivors and 7 deaths. Two patients who were still alive received surgery, chemotherapy. Results：By radiotherapy, and the survival time was 27 and 60 months, respectively. Five patients received surgery plus chemotherapy, and the survival time was 24, 25, 25, and 34 months, respectively, and the other one survived for 48 months by the end of the study. Four patients only received chemotherapy, and the survival time was 6(by the end of study), 12, 13, and 15 months, respectively. Conclusions：Multidisciplinary treatments using surgery, chemotherapy, and radiotherapy would improve the survival of DSRCT patients.