Sparing strategy does not compromise prognosis in pediatric localized XXXX : experience of the International Society of Pediatric Oncology, Malignant Mesenchymal Tumors (SIOP-MMT) Working Group.

@entity1 @entity5 @entity1352 @entity2802 @entity204 @entity1349
BACKGROUND: The aim of this analysis was to identify if the modified indications of radiotherapy (RT) or radical surgery compromised survival in pediatric @entity1352 ( @entity1352 ). PROCEDURE: @entity1 with non-metastatic @entity1352 , prospectively enrolled in three trials, were analyzed. After primary surgery or biopsy, they received chemotherapy. RT was planned after chemotherapy in @entity1 who had not achieved a complete response (CR). The considered outcome was 5-year @entity1349 ( @entity1349 ) and event-free survival (EFS). RESULTS: Eighty-eight @entity1 were identified. @entity5 were mainly located in limbs (66%). The first-line therapy for 65 @entity1 was primary resection. Of the 49 @entity1 who had gross @entity5 resection, 43 received adjuvant chemotherapy, and 8 had RT. All of the 39 @entity1 with macroscopic residual disease received chemotherapy, then only surgery (n = 12)    RT (n = 22). The 5-year EFS and @entity1349 rates were 68% and 85%, respectively. The @entity2802 stage was a prognostic factor for relapse, whereas primary site of the @entity5 and @entity2802 stage were prognostic factors for @entity204 . CONCLUSIONS: Only 32% of survivors received RT. @entity1349 was similar to published data. Omission of RT may be considered in younger @entity1 , to limit the potential sequelae in @entity1 with @entity5 less than 5 cm in size initially submitted to marginal resection. This strategy may also be considered in initially unresected cases, when the @entity5 is resected at delayed surgery with microscopically free margins, and in @entity1 in complete remission after primary chemotherapy.
