PURPOSE: @entity2269 ( @entity2269 ) is a rare aggressive malignancy that occurs in a young population with a male predominance. We studied the clinical and pathological characteristics of @entity2269 and investigated the effects of multimodal therapy including aggressive surgical resection, induction chemotherapy, and external beam radiotherapy. METHODS: We retrospectively reviewed and analyzed our experience with 11 histologically proven cases of @entity2269 between March 2004 and October 2014. The clinical information, histological, immunohistochemistry and survival data of the @entity1 were collected. RESULTS: The median age at diagnosis was 31.4 years (range, 14-64 years) and nine (82%) of the @entity1 were males. The most common presenting complaint was @entity490 (72.7%). Surgical resection was attempted in five @entity1 and included macroscopic total resection in two @entity1 and debulking in three @entity1 . Six @entity1 underwent biopsy only. Eleven @entity1 received multiagent chemotherapy. Five @entity1 (45.5%) received radiotherapy. The median survival of @entity1 who underwent surgical resection was 34.5 months, whereas the @entity1 who underwent biopsy alone was 24.5 months (P<0.05). The median survival was 40.8 months in radiotherapy group, and 19.2 months in non-radiotherapy group (P<0.05). The 3-year progression-free survival rate was 27.2%. The median survival was 29 months, and the median time to local failure was 8.8 months. Cox regression analysis showed surgery and radiotherapy were highly significant in prolonging @entity1 survival. CONCLUSION: Multimodal therapy consists of combination of surgical resection, chemotherapy and radiotherapy results in improved survival in @entity1 with @entity2269 . For unresectable @entity2269 , we recommend radiotherapy combined with @entity5948 -based chemotherapy.

Results of multimodal treatment for XXXX of the abdomen and pelvis.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity5948 :: ('D018943', 'Chemical') :: ['anthracycline'] @entity2269 :: ('MESH:D058405', 'Disease') :: ['Desmoplastic small round cell tumor', 'DSRCT'] @entity490 :: ('MESH:D015746', 'Disease') :: ['abdominal pain']
@entity2269 :: (MESH:D058405,Disease) :: ['desmoplastic small round cell tumor']
