Rhabdomyosarcoma (RMS) tumors arise from mesenchymal tissue and represent half of pediatric sarcomas, which in turn make up 7% of pediatric tumors. Advances in local control therapy of RMS have improved outcomes after surgical resection of the primary tumor, either before or after induction chemotherapy, even in the setting of metastatic disease. The utilization of diagnostic core needle and sentinel node biopsy techniques for lymph node staging are becoming more widely used. Over the past several years, refinement of prognostic factors with adoption of fusion status instead of histology, and optimized risk stratification schemas have been developed to assure appropriate therapy. There have been efforts between North American and European surgical oncology cooperative groups to standardize the care we provide, and yet there are still some philosophical differences to overcome.
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