Treatment of Metastatic Childhood Soft Tissue Sarcoma

Standard treatment options for metastatic childhood soft tissue sarcoma include the following:

For treatment options, see the individual tumor type sections of the summary.

The prognosis for children with metastatic soft tissue sarcomas is poor.[1-6] In a prospective randomized trial, chemotherapy with vincristine, dactinomycin, doxorubicin, and cyclophosphamide, with or without dacarbazine, led to tumor responses in one-third of patients with unresectable or metastatic disease. However, the estimated 4-year survival rate was poor. Less than one-third of children survived.[6-8]

Targeted (stereotactic body) radiation therapy is an option for sites of metastasis, particularly the lung.[9] Targeted radiation therapy is also an option for local control or sites of metastasis, including the lungs, bones, and brain,[10,11] particularly in patients for whom the morbidity of resection is a concern or whose life expectancy is limited.[9]

In a prospective trial of children with metastatic soft tissue sarcoma, patients were randomly assigned to receive multiagent chemotherapy with or without the addition of bevacizumab.[12] There was no difference in event-free survival or overall survival between the two study arms.