Childhood Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment Option Overview
The role of adjuvant (postoperative) chemotherapy remains controversial. A meta-analysis of updated data from adult STS patients from all available randomized trials concluded that recurrence-free survival was better with adjuvant chemotherapy for patients with high-grade tumors larger than 5 cm. The largest prospective pediatric trial failed to demonstrate any benefit with adjuvant vincristine, dactinomycin, cyclophosphamide, and doxorubicin. In a European trial, adults with completely resected STS were randomly assigned to observation or adjuvant chemotherapy with ifosfamide and doxorubicin, using a relatively low dose of ifosfamide. Patients assigned to adjuvant chemotherapy had adjuvant radiation therapy delayed until the end of chemotherapy. Like most adult trials, a wide variety of histologies were eligible. Adjuvant chemotherapy was not associated with improved event-free survival or overall survival. The authors merged their patients with previously published series, including those from the European meta-analysis, and concluded that the results suggested a benefit for adjuvant chemotherapy.[Level of evidence: 1iiA]
Special Treatment Considerations for Children With STS
Many therapeutic strategies for children and adolescents with soft tissue tumors are similar to those for adult patients, although there are important differences. For example, the biology of the neoplasm in pediatric patients may differ dramatically from that of the adult lesion. Additionally, limb-sparing procedures are more difficult to perform in pediatric patients. The morbidity associated with radiation therapy, particularly in infants and young children, may be much greater than that observed in adults.
Improved outcomes with multimodality therapy in adults and children with STSs over the past 20 years has caused increasing concern about the potential long-term side effects of this therapy in children, especially when considering the expected longer life span of children versus adults. Therefore, to maximize tumor control and minimize long-term morbidity, treatment must be individualized for children and adolescents with nonrhabdomyosarcomatous STS. These patients should be enrolled in prospective studies that accurately assess any potential complications.
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