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

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Changes to This Summary (04/26/2016)

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 Figures 1, 2, and 3 depicting the distribution of nonrhabdomyosarcomatous soft tissue sarcomas by stage, histologic subtype, and tumor site (cited Ferrari et al. as reference 7).

Added text to state that a large, prospective, multinational Children's Oncology Group (COG) study (ARST0332) enrolled newly diagnosed patients younger than 30 years. Patients were assigned to treatment on the basis of their risk group (cited Spunt et al. as reference 47 and level of evidence 2A).

Added Figure 4 depicting the risk stratification and treatment assignment for the COG ARST0332 trial.

Added text about the treatment options for each arm of the COG ARST0332 trial.

Added text about the estimated 3-year event-free survival and overall survival rates for each arm of the COG ARST0332 trial.

Treatment of Newly Diagnosed Childhood Soft Tissue Sarcoma

Added text to state that the SARC023 trial is testing the combination of ganetespib, the heat shock protein inhibitor, and sirolimus, the mammalian target of rapamycin inhibitor, for the treatment of patients with unresectable or metastatic malignant peripheral nerve sheath tumors. Eligibility is restricted to patients aged 18 years and older.

Added text to state that the disease course for perivascular epithelioid cell differentiation (PEComas) may be indolent.

Added text to state that treatment options have not been defined for PEComas. Treatment may include surgery or observation followed by surgery when the tumor is large (cited Alaggio et al. as reference 175).

Added Treatment options under clinical evaluation as a new subsection.

The Angiosarcoma of the soft tissue subsection was extensively revised.

The Epithelioid hemangioendothelioma subsection was extensively revised.

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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