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Diagnosis and Treatment of Desmoplastic Small Round Cell Tumor

SUN Peng;XU Yu-qing;The Second Affiliated Hospital of Haerbin Medical University;  
Desmoplastic small round cell tumor(DSRCT) is a very rare,highly aggressive malignant soft tissue sarcoma usually occurring in abdominal and pelvic cavity of male adolescents. The specific organs or tissue types of origin are not identified. The disease is lack of characteristic clinical manifestations.Its diagnosis is based on clinicopathological features,immune phenotype and molecular genetics. DSRCT is associated with a unique chromosomal translocation t(11,22)(P13;q12),generating EWS-WT1 fusion gene. Reverse transcriptase-polymerase chain reaction(RT-PCR),fluorescence in situ hybridization and Southern blot are used to detect the fusion gene and to indentify the DSRCT. The prognosis of DSRCT is very poor,and the median survival time ranges from 17 to 25 months. The optimal management of DSRCT at present is not consentient. Multi-disciplinary comprehensive treatment including debulking surgery,chemotherapy,radiation,and hyperthermal intraperitoneal chemotherapy(HIPEC) may prolong survival time to a certain extent,however,the overall effect is poor. Emerging therapeutic measures such as targeted therapy and immune therapy need to be further studied.
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