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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.

About the National Comprehensive Cancer Network

The National Comprehensive Cancer Network® (NCCN®), a not-for-profit alliance of 27 leading cancer centers, is dedicated to improving the quality and effectiveness of care provided to patients with cancer. Through the leadership and expertise of clinical professionals at NCCN Member Institutions, NCCN develops resources that present valuable information to the numerous stakeholders in the health care delivery system. As the arbiter of high-quality cancer care, NCCN promotes the importance of continuous quality improvement and recognizes the significance of creating clinical practice guidelines appropriate for use by patients, clinicians, and other health care decision-makers. The primary goal of all NCCN initiatives is to improve the quality, effectiveness, and efficiency of oncology practice so patients can live better lives. For more information, visit NCCN.org.

The NCCN Member Institutions are:

  • Fred & Pamela Buffett Cancer Center
  • Case Comprehensive Cancer Center/University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute
  • City of Hope Comprehensive Cancer Center
  • Dana-Farber/Brigham and Women's Cancer Center | Massachusetts General Hospital Cancer Center
  • Duke Cancer Institute
  • Fox Chase Cancer Center
  • Huntsman Cancer Institute at the University of Utah
  • Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance
  • The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins
  • Robert H. Lurie Comprehensive Cancer Center of Northwestern University
  • Mayo Clinic Cancer Center
  • Memorial Sloan Kettering Cancer Center
  • Moffitt Cancer Center
  • The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute
  • Roswell Park Cancer Institute
  • Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine
  • St. Jude Children's Research Hospital/The University of Tennessee Health Science Center
  • Stanford Cancer Institute
  • University of Alabama at Birmingham Comprehensive Cancer Center
  • UC San Diego Moores Cancer Center
  • UCSF Helen Diller Family Comprehensive Cancer Center
  • University of Colorado Cancer Center
  • University of Michigan Comprehensive Cancer Center
  • The University of Texas MD Anderson Cancer Center
  • University of Wisconsin Carbone Cancer Center
  • Vanderbilt-Ingram Cancer Center
  • Yale Cancer Center/Smilow Cancer Hospital