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Childhood Soft Tissue Sarcoma Treatment (PDQ®)

Changes to This Summary (01/29/2015)

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

Added text to state that although rare, nonrhabdomyosarcomatous soft tissue sarcomas (STSs) can arise primarily in brain tissue and are treated according to the histiotype (cited Benesch et al. as reference 20).

Added text about the use of biopsy to diagnose nonrhabdomyosarcomatous STSs. Also added text to state that excisional biopsy of the lesion is only appropriate for small superficial lesions (cited Coffin et al. and Smith et al. as references 25 and 26, respectively).

Added text to state that in children with unplanned resection of nonrhabdomyosarcomatous STSs, primary re-excision is frequently recommended because many patients will have tumor present in the re-excision specimen (cited Chui et al. and Cecchetto et al. as references 27 and 28, respectively).

Revised text to state that infantile fibrosarcoma, presenting in infants and children younger than 5 years, has an excellent prognosis given that the tumor is highly chemosensitive and surgery alone can cure a significant number of these patients.

Treatment Option Overview

Added Treatment Options Under Clinical Evaluation as a new subsection.

Treatment of Newly Diagnosed Childhood Soft Tissue Sarcoma

Added text to state that inactivating mutations of SUZ12 have been described in malignant peripheral nerve sheath tumors and are absent in neurofibromas (cited Zhang et al. as reference 75).

Added text to state that male gender and non-Hispanic white race are features associated with favorable prognosis (cited Amirian et al. as reference 79).

Added text to state that a synovial sarcoma may arise in the heart or pericardium (cited Wang et al. as reference 131).

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.

  • Updated: January 29, 2015