Because of the rarity of pediatric nonrhabdomyosarcomatous soft tissue sarcomas (NRSTSs), all children, adolescents, and young adults with these tumors should have their treatment planned by a multidisciplinary team composed 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.

Every attempt should be made to resect the primary tumor with negative margins before or after chemotherapy. The timing of surgery depends on an assessment of the feasibility and morbidity of surgery. Involvement of a surgeon with special expertise in the resection of soft tissue sarcomas in the decision is highly desirable. Sentinel lymph node mapping is employed at some centers to identify the regional nodes that are the most likely to be involved, though its contribution has not been clearly defined.[1,2] If the original operation failed to achieve pathologically negative tissue margins, a second procedure should be performed to obtain clear, but not necessarily wide margins.[3-7] When the initial operation was done without the knowledge that cancer was present, a re-excision of the affected region should always be considered, even in the absence of a mass on magnetic resonance imaging.[8] When there is concern about the adequacy of the surgical margin, radiation therapy is indicated.[9] This is particularly important in high-grade tumors with tumor margins less than 1 cm.[10,11] Thus, by using these two treatment modalities, local control of the primary tumor can be achieved in more than 80% of patients.[12,13] Although combined surgery and radiation therapy have dramatically improved outcome in adults and children with soft tissue sarcomas over the past 20 years,[9] the morbidity of high-dose radiation therapy is of concern in infants and young children with these tumors.[14] Brachytherapy and intraoperative radiation may be applicable in select situations.[13,15,16] Preoperative radiation therapy has been associated with excellent local control rates [17-19] but has been associated with an increased rate of wound complications in adults.[20] Patients in the pediatric age group with unresected NRSTS have a poor outcome. Only about one-third of patients treated with multimodality therapy remain disease free.[20,21]

Therapeutic strategies for children and adolescents with soft tissue tumors are similar to those for adult patients, though there are important differences. For example, the biology of the pediatric form of the neoplasm may differ dramatically from that of the adult lesion. Limb-sparing procedures are more difficult to perform in pediatric patients. In addition, the morbidity of radiation therapy in young children may be much greater than that observed in adults. Lastly, the concern regarding potential long-term side effects of combined modality therapy (radiation, surgery, and chemotherapy) is greater for children, whose survival may be much longer than that of adults. Therefore, to maximize tumor control and minimize long-term morbidity, treatment must be individualized for children and adolescents with nonrhabdomyosarcomatous soft tissue tumors. These patients should be enrolled in prospective studies that accurately assess any potential complications.[22]

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.[23] The largest prospective pediatric trial failed to demonstrate any benefit with adjuvant vincristine, dactinomycin, cyclophosphamide, and doxorubicin.[12] 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.[24-28] A German trial suggested a benefit for adjuvant chemotherapy in children with synovial sarcoma.[29] A meta-analysis also suggested that chemotherapy may provide benefit.[30] 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.

References

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