BACKGROUND: @entity2875 [RME] is the most common @entity242 . Whereas the prognosis of localized @entity980 has improved, it remains poor for metastatic disease. METHODS: We analyzed RME- @entity1 with isolated pulmonary @entity3 [PRME] treated in four consecutive CWS-trials. Treatment included multiagent chemotherapy and local treatment of the @entity5 . Therapy of lung @entity3 after induction chemotherapy depended on response and individual decisions. RESULTS: Twenty-nine @entity1 <21 years had PRME. Their median age was six years, the median follow-up nine years. Twenty-eight @entity1 had their @entity5 located in an unfavorable site and 22 of the primaries were >5 cm. In addition to conventional chemotherapy, seven @entity1 received high-dose treatment and eight @entity1 oral metronomic chemotherapy. The lung @entity3 were in remission after induction chemotherapy in 22 individuals. 19 @entity1 received no local treatment of @entity3 ; 3 @entity1 had pulmonary metastasectomy and lung radiation was administered to 9 individuals. In total, 24/29 @entity1 achieved a complete remission [CR]. Actuarial 5-year event-free and overall survival for all @entity1 was 37.9    18% and 48.7    18%, respectively; it was 45.8    20% and 58.3    20% for the 24 @entity1 who achieved a CR. Local treatment of @entity3 had no impact on the failure pattern. Younger age, good response, achievement of CR and maintenance-treatment were favorable prognostic factors in univariate analysis. CONCLUSIONS: @entity1 with PRME have a fair prognosis. Local treatment of @entity3 did not improve outcome in our sample. Metronomic treatment may be an attractive option for PREM- @entity1 .

@entity2875 with XXXX confined to the lungs: report from the CWS Study Group.

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'Children'] @entity242 :: ('MESH:D012509', 'Disease') :: ['pediatric soft tissue sarcoma'] @entity5 :: ('MESH:D009369', 'Disease') :: ['primary tumor'] @entity3 :: ('MESH:D009362', 'Disease') :: ['metastases'] @entity2875 :: ('MESH:D018233', 'Disease') :: ['Embryonal rhabdomyosarcoma'] @entity980 :: ('MESH:D012208', 'Disease') :: ['rhabdomyosarcoma']
@entity3 :: (MESH:D009362,Disease) :: ['metastases']
