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Childhood Soft Tissue Sarcoma Treatment (PDQ®)

Health Professional Version

Changes to This Summary (09/08/2014)

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 Smith et al. as reference 1.

Revised text to state that between 1975 and 2010, childhood cancer mortality decreased by more than 50%.

Treatment of Newly Diagnosed Childhood Soft Tissue Sarcoma

Added text to state that a retrospective review of children who underwent surgery for desmoid tumors at the St. Jude Children’s Research Hospital reported no correlation between surgical margins and risk of recurrence (cited Soto-Miranda et al. as reference 20).

Added Sulkowski et al. as reference 36.

Added text to state that in a series of nine patients, four patients who had a complete resection achieved continuous remission, three patients who had residual disease had a high recurrence rate and achieved continuous remission, and one patient who had metastatic disease responded to multiagent chemotherapy (cited Mehta et al. as reference 55 and level of evidence 3iiA).

Added text to state that a series of 51 pediatric patients aged 0 to 21 years with alveolar soft part sarcoma found an overall survival (OS) rate at 10 years of 78% and an event-free survival (EFS) rate of about 63%. Patients with localized disease had a 10-year OS of 87%, and the 14 patients with metastases at diagnosis had a 10-year OS of 44%. Only 3 of 18 patients with measurable disease had a response to conventional antisarcoma chemotherapy, but two of four patients treated with sunitinib had a partial response (cited Orbach et al. as reference 85 and level of evidence 3iiiA). Also added text to state that there have been sporadic reports of objective responses to interferon-alpha and bevacizumab; and that in a phase II trial of cediranib, 15 of 43 patients with metastatic alveolar soft part sarcoma had a partial response (cited Kummar et al. as reference 88 and level of evidence 3iiDiv).

Added text about the NCT00942877 and NCT01391962 trials as treatment options under clinical evaluation for alveolar soft part sarcoma.

Treatment of Metastatic Childhood Soft Tissue Sarcoma

Revised Table 9 to include cediranib as an agent with selective activity against the alveolar soft part sarcoma subtype.

Treatment of Progressive/Recurrent Childhood Soft Tissue Sarcoma

Added text to state that pazopanib has been approved for use in recurrent soft tissue sarcoma; the clinical trial that was used to obtain approval was limited to adults and demonstrated disease stabilization and prolonged time to progression, but it did not demonstrate improved overall survival (cited van der Graaf as reference 6).

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: September 8, 2014