NCCN Updates Dermatofibrosarcoma Protuberans Guidelines

JENKINTOWN, Pa., September 11, 2007 — The National Comprehensive Cancer Network (NCCN) announces important updates to the NCCN Dermatofibrosarcoma Protuberans (DFSP) Guidelines. DFSP is a rare, low-grade sarcoma characterized by a t(17;22) chromosomal translocation resulting in the over-expression of platelet-derived growth factor receptor β(PDGFRB). This abnormal proliferation of cells can quickly grow into a malignant tumor.

In a phase II open-label trial—Study B2225, the largest study involving patients with DFSP)—imatinib mesylate, an inhibitor of PDGFRB, was found to be safe and effective in the treatment of patients with locally advanced and metastatic DFSP tumors containing t(17;22) translocation. Based on the results of this study and five other published case reports, the FDA approved imatinib mesylate for the treatment of adult patients with unresectable, recurrent and/or metastatic DFSP.

In response to the favorable results and the FDA approval, the NCCN DFSP Guidelines now include imatinib mesylate (Gleevec®, Novartis Pharmaceuticals) as an option for patients with DFSP with the following indications:

     (i) positive surgical margins following re-resection, if further resection is not feasible
     (ii) recurrent disease, if additional resection would lead to unacceptable functional or cosmetic outcomes
     (iii) metastatic disease.

Study B2225 also showed that patients with DFSP lacking t(17;22) may not respond to imatinib. As a result, the guidelines suggest that molecular analysis of a tumor using cytogenetics may be useful prior to the institution of imatinib therapy.

The NCCN Clinical Practice Guidelines in Oncology™ are widely recognized and applied as the standard of care in oncology in the United States in both the community and the academic practice settings. These guidelines are developed and continually updated through an evidence-based process with explicit review of the scientific evidence by multidisciplinary panels of expert physicians from NCCN Member Institutions. The most recent version of this and all the guidelines are available free of charge at www.nccn.org.