PubMed · 13972165
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P J PONTINEN. 1963. [Dermatofibrosarcoma protuberans].. https://pubmed.ncbi.nlm.nih.gov/13972165/
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In 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.
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A 60-year-old woman had a slow-growing cutaneous nodule of her left brow. After initial excisional biopsy, the lesion recurred within 1 week. After tissue diagnosis was confirmed, the recurrent mass was excised under frozen section control and the defect was reconstructed with a forehead flap. This unusual tumor involved the brow and eyelid, down to the level of the orbital septum. The patient remains free of disease after 20 months.