Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (12/06/2016)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

General Information About Childhood Soft Tissue Sarcoma

Added Wang et al. as reference 43.

Treatment of Newly Diagnosed Childhood Soft Tissue Sarcoma

Added text to state that in a subsequent study, only one of seven patients with group II disease progressed during observation; that patient achieved complete remission with chemotherapy (cited Orbach et al. as reference 67 and level of evidence 3iiA).

Revised text to state that three studies of patients with infantile fibrosarcoma suggest that an alkylator-free regimen is effective and should be used as the first treatment choice in patients with macroscopic disease.

Added text to state that a single case report described a patient aged 2 months who was diagnosed in utero with infantile fibrosarcoma; the infant was initially treated with chemotherapy. At disease progression, treatment with the vascular endothelial growth factor receptor inhibitor pazopanib resulted in tumor response (cited Yanagisawa et al. as reference 76).

Added targeted therapy as a treatment option for inflammatory myofibroblastic tumor.

Added text to state that features with an unfavorable prognosis include high grade, deep tumor location, locally advanced stage at diagnosis, and macroscopically incomplete resection (cited Valentin et al. as reference 118).

Added text to state that in the French Sarcoma Group study, neurofibromatosis type 1 was associated with other adverse prognostic features, but was not an independent predictor of poor outcome.

Revised text to state that desmoplastic small round cell tumor most frequently involves the abdomen, pelvis, or tissues around the testes, but it may occur in the kidney (cited Wang et al. as reference 157).

Added text to state that a review of 84 patients with localized synovial sarcoma who had information on fusion status and histologic grading found no difference in overall survival (OS) according to these criteria. However, for tumor size at diagnosis, the study showed that patients with tumors between 5 cm and 10 cm had a worse prognosis than those with smaller tumors, and patients with tumors larger than 10 cm had even worse OS (cited Stegmaier et al. as reference 210 and level of evidence 3iiiA).

Added text about a prospective study from the European Pediatric Soft Tissue Sarcoma Study Group of patients younger than 21 years with synovial sarcoma, their risk groups, treatment assignments, and outcomes (cited Ferrari et al. as reference 224 and level of evidence 3iiA).

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

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