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lüll Dermatofibrosarcoma protuberans Mendenhall WM; Zlotecki RA; Scarborough MTCancer 2004[Dec]; 101 (11): 2503-8In the current review, the authors set out to discuss the natural history and treatment of dermatofibrosarcoma protuberans (DFSP), a rare indolent cutaneous tumor. Approximately 10-15% of all DFSPs contain areas of fibrosarcoma (DFSP-FS), and such cases tend to exhibit more aggressive behavior. The optimal treatment for DFSP is resection with wide margins; the likelihood of local control associated with this procedure exceeds 90%. The probability of regional or distant metastases is = 5%. Patients with positive or close surgical margins have an elevated risk of local recurrence after resection alone; however, postoperative radiotherapy results in local control rates of >/= 85% in such patients. Postoperative radiotherapy also is indicated in the rare event that a patient has unresectable macroscopic disease. Experience with the use of radiotherapy alone to treat macroscopic disease is limited.|*Neoplasm Recurrence, Local[MESH]|Dermatofibrosarcoma/*pathology/radiotherapy/surgery[MESH]|Diagnosis, Differential[MESH]|Disease Progression[MESH]|Humans[MESH]|Prognosis[MESH]|Radiotherapy, Adjuvant[MESH]|Risk Factors[MESH]|Skin Neoplasms/*pathology/radiotherapy/*surgery[MESH] |