Revision made to NCCN treatment guide for rare sarcoma

Imatinib (Gleevec, Novartis) is now included as a treatment option for patients with dermatofibrosarcoma protuberans (DFSP) as part of newly revised treatment guidelines from the National Comprehensive Cancer Network (NCCN). In a phase II trial (Study B2225), the inhibitor of platelet-derived growth factor receptor β was shown to be safe and effective in treating tumors containing t(17;22) chromosomal translocation, a characteristic of DFSP that leads to abnormal proliferation of cells that develop into a malignancy. The new guidelines recommend imatinib as an option in patients with positive surgical margins following resection, recurrent disease, or metastatic disease. The new guide points out that imatinib may not benefit patients with DFSP who lack the t(17;22) translocation and, therefore, molecular analysis of the tumor using cytogenetics is suggested prior to initiating therapy. The FDA approved imatinib for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP in October 2006.

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