Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Because of the rarity of pediatric nonrhabdomyosarcomatous soft tissue sarcomas (NRSTSs), all children, adolescents, and young adults with these tumors should have their treatment coordinated by a multidisciplinary team comprised of pediatric oncologists, surgeons, and radiotherapists. To better define the tumors' natural history and response to therapy, children with rare neoplasms should be considered for entry into national or institutional treatment protocols.
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Every attempt should be made to resect the primary tumor with negative margins before or after chemotherapy. Involvement of a surgeon with special expertise in the resection of soft tissue sarcomas in the decision is highly desirable. The timing of surgery depends on an assessment of the feasibility and morbidity of surgery. If the initial surgery fails to achieve pathologically negative tissue margins or if the initial surgery was done without the knowledge that cancer was present, a re-excision of the affected area should be performed to obtain clear, but not necessarily wide, margins.[1,2,3,4,5] This surgical tenet is true even if no mass was detected by magnetic resonance imaging after initial surgery.; [Level of evidence: 3iiA] Regional lymph node metastases at diagnosis are unusual and appear most likely with epithelioid and clear cell sarcomas. Sentinel lymph node mapping is employed at some centers to identify the regional nodes that are the most likely to be involved, though its widespread contribution to the staging and management of these tumors has yet to be clearly defined.[9,10,11]
Radiation therapy is indicated for patients with inadequate surgical margins and for larger, high-grade tumors. This is particularly important in high-grade tumors with tumor margins less than 1 cm.[13,14]; [Level of evidence: 3iiDiv] When using both surgery and radiation therapy, local control of the primary tumor can be achieved in more than 80% of patients.[16,17] Brachytherapy and intraoperative radiation may be applicable in select situations.[17,18,19]; [Level of evidence: 3iiiDii] Preoperative radiation therapy has been associated with excellent local control rates,[21,22,23] but has been associated with an increased rate of wound complications in adults. Pediatric patients with unresected NRSTS have a poor outcome. Only about one-third of patients treated with multimodality therapy remain disease free.[24,25]
The role of adjuvant (postoperative) chemotherapy remains controversial. A meta-analysis of updated data from adult soft tissue sarcoma patients from all available randomized trials concluded that recurrence-free survival was better with adjuvant chemotherapy. The largest prospective pediatric trial failed to demonstrate any benefit with adjuvant vincristine, dactinomycin, cyclophosphamide, and doxorubicin. Synovial sarcoma appears to be more sensitive to chemotherapy than many other soft tissue sarcomas, and children with synovial sarcoma seem to have a better prognosis.[28,29,30,31,32] A German trial suggested a benefit for adjuvant chemotherapy in children with synovial sarcoma. A meta-analysis also suggested that chemotherapy may provide benefit. Many treatment centers advocate adjuvant chemotherapy following resection of synovial sarcoma in children and young adults; unequivocal proof of the value of this strategy from prospective, randomized clinical trials is lacking.