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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:

  • Combination therapy using chemotherapy, radiation therapy, and surgical resection of pulmonary metastases.

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. 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 following the use of focused radiation therapy in adults.[9] Formal segmentectomy, lobectomy, and mediastinal lymph node dissection are unnecessary.[10]

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

AgentSoft Tissue Sarcoma Subtype
Sunitinib[11,12]Alveolar soft part sarcoma
Sunitinib[13]Solitary fibrous tumor
Sirolimus[14]Perivascular epithelioid cell tumor (PEComa)
Crizotinib[15]Inflammatory myofibroblastic tumor
ImatinibTenosynovial giant cell tumor
Imatinib[17]Dermatofibrosarcoma protuberans

The Children's Oncology Group COG-ARST0332 trial entered 552 eligible patients younger than 30 years with localized and metastatic nonrhabdomyosarcoma STSs from 2007 until 2012. The study is now closed and awaits analysis as the first risk-based treatment program for nonrhabdomyosarcoma STS in this age group.

The following are examples of national and/or institutional clinical trials that are currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.

  • NCT00942877 (Phase II Study of Cediranib [AZD2171] in Patients With Alveolar Soft Part Sarcoma): This study is designed for individuals aged 18 years and older who have been diagnosed with alveolar soft part sarcoma. AZD2171 blocks the creation of new blood vessels. Patients will take AZD2171 orally once a day, every day, for the duration of the study. The treatment will be given in 28-day cycles.
  • COG-ADVL0921 (MLN8237 in Treating Young Patients With Recurrent or Refractory Solid Tumors or Leukemia): This phase II study is evaluating the side effects and efficacy of MN8237, an aurora A kinase inhibitor. Patients receive oral MN8237 once daily on days 1 to 7. Treatment repeats every 21 days for up to 35 courses in the absence of disease progression or unacceptable toxicity.
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