BACKGROUND: @entity2269 (DSRCT or DSCT) are @entity5 and early adulthood. There are few reported series to guide clinical therapy. This study correlates survival with treatment variables, including aggressive surgical debulking. METHODS: Thirty-two @entity1 with documented DSRCT received treatment at our institution. Demographic, clinical, and treatment variables were correlated with progression-free survival using log-rank statistics. RESULTS: Thirty @entity1 were male (96%), and two were female (4%), with a median age at diagnosis of 22 years. The primary site of disease in 97% of cases was the abdomen or pelvis. Twenty-nine @entity1 (91%) had extensive disease involving peritoneal surfaces, lymph nodes, or discontinuous organs. All 32 @entity1 received systemic chemotherapy. Fifteen (47%) underwent @entity5 debulking greater than 90% at diagnosis or during therapy. A complete or very good response to therapy occurred in 13 @entity1 , and depended on surgical @entity66 in all. Thirteen @entity1 remained progression-free, but three of these @entity1 died from treatment @entity137 . Improved survival was correlated with a complete or very good partial response to multimodality therapy, surgical debulking of more than 90% either before or after chemotherapy, and use of the P6 protocol. CONCLUSIONS: DSRCT is an aggressive @entity5 that occurs predominantly in young males. Improved survival is correlated with intense chemotherapy and aggressive resection.

XXXX : prognostic indicators and results of surgical management.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity137 :: ('MESH:D064420', 'Disease') :: ['toxicity'] @entity66 :: ('MESH:D004194', 'Disease') :: ['removal of bulk disease'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'rare aggressive cancers of adolescence', 'cancer'] @entity2269 :: ('MESH:D058405', 'Disease') :: ['Desmoplastic small round cell tumors']
@entity2269 :: (MESH:D058405,Disease) :: ['Desmoplastic small round cell tumors']
