BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a very rare, low-grade malignant tumor. Local recurrence is frequent after incomplete resection either because of false diagnosis or inadequate standard surgical excision. This study evaluated the clinical outcome of micrographic surgery with paraffin sectioning in the therapy of DFSP. METHODS: Medical charts of 22 patients with histologically confirmed DFSP treated at our institution between 1987 and 2002 were reviewed. RESULTS: For 13 patients with primary and 9 with recurrent disease, tumor-free excision margins could be achieved, and all patients remained free of local recurrence during a mean follow-up of 54 months. CONCLUSIONS: The results of our study and a review of the literature confirm the successful and recurrence-free treatment of DFSP with micrographic surgery as the treatment of choice for this locally invasive tumor. Particularly in recurrent lesions, we recommend the use of paraffin sectioning and three-dimensional histological evaluation as an accurate additional tool for treatment optimization.
BACKGROUND:Dermatofibrosarcoma protuberans (DFSP) is a very rare, low-grade malignant tumor. Local recurrence is frequent after incomplete resection either because of false diagnosis or inadequate standard surgical excision. This study evaluated the clinical outcome of micrographic surgery with paraffin sectioning in the therapy of DFSP. METHODS: Medical charts of 22 patients with histologically confirmed DFSP treated at our institution between 1987 and 2002 were reviewed. RESULTS: For 13 patients with primary and 9 with recurrent disease, tumor-free excision margins could be achieved, and all patients remained free of local recurrence during a mean follow-up of 54 months. CONCLUSIONS: The results of our study and a review of the literature confirm the successful and recurrence-free treatment of DFSP with micrographic surgery as the treatment of choice for this locally invasive tumor. Particularly in recurrent lesions, we recommend the use of paraffin sectioning and three-dimensional histological evaluation as an accurate additional tool for treatment optimization.
Authors: Piotr Rutkowski; Martine Van Glabbeke; Cathryn J Rankin; Wlodzimierz Ruka; Brian P Rubin; Maria Debiec-Rychter; Alexander Lazar; Hans Gelderblom; Raf Sciot; Dolores Lopez-Terrada; Peter Hohenberger; Allan T van Oosterom; Scott M Schuetze Journal: J Clin Oncol Date: 2010-03-01 Impact factor: 44.544