PubMed · 15419166
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C MOPPER, H PINKUS. 1950. Dermatofibrosarcoma protuberans.. https://doi.org/10.1093/ajcp%2F20.2.171
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Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive, rarely metastatic, spindle cell tumor. It is most commonly found on the trunk and rarely on the head and neck. Recurrence has been found to be more common in the head and neck region, which may be attributable to delayed patient presentation or a reluctance for wide excision in that region. The authors report a rare case of DFSP presenting as a large mass of the face. Surgical treatment required aggressive local resection of the tumor, including the underlying maxillary antrum. The soft tissue was cleared by Mohs technique. More than one procedure was required to obtain clear bone margins. Adjuvant radiation therapy was used to gain local control. Reconstruction was performed using a cheek advancement flap, as well as a forehead flap and split-thickness graft to the donor site.
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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