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    Childhood Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Metastatic Childhood Soft Tissue Sarcoma

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

    The prognosis for children with metastatic STSs is poor,[1,2,3,4,5,6] and these children should receive combined treatment with chemotherapy, radiation therapy, and surgical resection of pulmonary metastases. 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. The estimated 4-year survival rate, however, was poor, with fewer than one-third of children surviving.[6,7,8]

    Pulmonary Metastases

    Children with isolated pulmonary metastases should undergo a surgical procedure in an attempt to resect all gross disease. For patients with multiple or recurrent pulmonary metastases, additional surgical procedures can be performed if the morbidity is deemed acceptable. In a retrospective review, patients with synovial sarcoma and pulmonary metastases for whom it was possible to completely resect all metastatic lung lesions had better survival than did patients for whom it was not possible to achieve complete resections.[9][Level of evidence: 3iiiA] An alternative approach is focused radiation therapy (fractionated stereotactic radiation therapy), which has been successfully used in adults to sterilize lesions. The estimated 5-year survival rate after thoracotomy for pulmonary metastasectomy has ranged from 10% to 58% in adult studies. Emerging data suggest a similar outcome after the administration of focused radiation therapy in adults.[10] Formal segmentectomy, lobectomy, and mediastinal lymph node dissection are unnecessary.[11]

    Treatment Options Under Clinical Evaluation

    The following agents are being studied for the treatment of certain metastatic STSs:

    Table 9. Agents With Selective Activity Against Subtypes of Soft Tissue Tumors

    Agent Soft Tissue Sarcoma Subtype
    Sunitinib[12,13] Alveolar soft part sarcoma
    Cediranib[14] Alveolar soft part sarcoma
    Sunitinib[15] Solitary fibrous tumor
    Sirolimus[16] Perivascular epithelioid cell tumor (PEComa)
    Crizotinib[17] Inflammatory myofibroblastic tumor
    Imatinib Tenosynovial giant cell tumor
    Imatinib[18] Chordoma
    Imatinib[19] Dermatofibrosarcoma protuberans

    Current Clinical Trials

    Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with metastatic childhood soft tissue sarcoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

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