UNASSIGNED: @entity980 ( @entity980 ) represents the most common soft tissue @entity242 in @entity1 and @entity1 and the third most common pediatric solid @entity5 , accounting for 5% to 15% of all childhood solid @entity5 . Of these, 15% to 20% arise from the genitourinary tract, with the most common sites originating from the prostate, bladder, and paratesticular regions, followed by the vagina and uterus. Although upfront radical surgery was used at the initiation of Intergroup @entity980 Study-I (1972-1978), the treatment paradigm has shifted to include initial biopsy with the goal of organ preservation, systemic chemotherapy for all @entity1 , and local control involving surgical resection with or without radiation therapy for most @entity1 . Collaborative group clinical trials have led to dramatic improvement in survival rates from 1960 to 1996 among @entity1 with low- or intermediate-risk disease; however, outcomes appear to have plateaued in more recent years, and the prognosis for @entity1 with metastatic or relapsed/refractory disease remains poor. Current management goals include minimizing @entity137 while maintaining the excellent outcomes in low-risk disease, as well as improving outcomes in @entity1 with intermediate- and high-risk disease. Advances in genetic analysis have allowed further refinement in risk stratification of @entity1 . Perhaps the most significant recent development in @entity980 research was the discovery of an association of @entity3943 ( @entity133 ) with translocations t(2;13) and t(1;13). Translocation fusion-positive @entity5 comprise 80% of @entity133 and are more aggressive. Fusion-negative @entity133 may have a clinical course similar to embryonal @entity980 . Future @entity1 * s Oncology Group @entity242 studies will likely incorporate fusion status into risk stratification and treatment allocation. Newer radiotherapy modalities hold promise for providing local control of disease while minimizing morbidity. The addition of traditional cytotoxic chemotherapeutic agents does not seem to improve outcomes in high-risk @entity1 . Ultimately, the most substantial progress may arise from further elucidation of genetic and molecular pathways involved in @entity980 @entity5 formation in an effort to identify novel, targeted therapeutic approaches.

Future directions in risk stratification and therapy for advanced pediatric XXXX .

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'Children', 'infants'] @entity137 :: ('MESH:D064420', 'Disease') :: ['toxicity'] @entity242 :: ('MESH:D012509', 'Disease') :: ['sarcoma'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'tumors'] @entity133 :: ('MESH:D002282', 'Disease') :: ['ARMS'] @entity980 :: ('MESH:D012208', 'Disease') :: ['RMS', 'Rhabdomyosarcoma'] @entity3943 :: ('MESH:D018232', 'Disease') :: ['alveolar RMS']
@entity980 :: (MESH:D012208,Disease) :: ['genitourinary rhabdomyosarcoma']
