Present-day concepts in the management of sarcomas in children

1. NRSTS tumors represent 20-20% of all soft tissue sarcomas. In only the extremity primary are both RMS and NRSTS occurring with almost equal frequency. 2. Modern diagnostic imaging studies are invaluable to staging and preoperative planning. 3. The most important need is to standardize biopsy techniques, since an ill-conceived biopsy has an adverse effect on the definitive surgical procedure. 4. Standardization of surgical procedures is necessary to adequately define extent of local resection varying with site, type of tumor, and its biological behavior. Less radical procedures do not seem to have had an adverse effect in local control or survival as is evidenced in RMS of the orbit, vagina, and bladder. 5. Though complete surgical extirpation is the treatment of choice in RMS, incomplete surgical excision leaving microscopic disease can result in adequate local control following chemotherapy and radiation therapy. However, less than optimum responses to chemotherapy in the NRSTS makes it imperative that efforts be made to completely resect these lesions. 6. It is in the realm of initially unresectable primary lesion that the surgical oncologist's role be refined. Consideration here includes: (a) use of newer surgical techniques such as laser, or free microvascular grafts, (b) protocols to define the optimum timing of delayed surgery following preoperative chemotherapy with or without radiation therapy.