Dermatofibrosarcoma Protuberans William M. Mendenhall, M.D. 1 Robert A. Zlotecki, M.D., Ph.D. 1 Mark T. Scarborough, M.D. 2 1 Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, Florida. 2 Department of Orthopedic Surgery, University of Florida College of Medicine, Gainesville, Florida. Address for reprints: William M. Mendenhall, M.D., Department of Radiation Oncology, University of Florida Health Science Center, P.O. Box 100385, Gainesville, FL 32610-0386; Fax: (352) 265-0759; E-mail: [email protected]fl.edu Received August 5, 2004; accepted August 18, 2004. 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 treat- ment 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 mac- roscopic disease is limited. Cancer 2004;101:2503– 8. © 2004 American Cancer Society. KEYWORDS: dermatofibrosarcoma protuberans, resection, positive or close mar- gins, radiotherapy. D ermatofibrosarcoma protuberans (DFSP) is a rare monoclonal cutaneous soft tissue sarcoma that was first described by Taylor in 1890. 1 Chang et al., 2 reporting on 60 patients with DFSP who were treated at the University of Illinois–Chicago (Chicago, IL) between 1968 and 2001, estimated that approximately 100 patients with soft tissue sarcoma (any type) were treated each year at their institution; thus, DFSP accounted for approximately 1.8% of all soft tissue sarco- mas encountered. Approximately 85–90% of all DFSPs are low-grade lesions; the remainder contain a high-grade fibrosarcomatous com- ponent, which accounts for 5% of the tumor volume, and are considered to be intermediate-grade lesions (known as ‘DFSP-FSs’). 3 DFSPs arise as pink or violet-red plaques, and the surrounding skin may be telangiectatic. 4 These lesions typically are fixed to the dermis but move freely over deeper-lying tissue, and they do not exhibit a nodular growth pattern until late in their course. Fixation to more deeply seated structures often is observed in advanced and/or recur- rent cases of DFSP. 4 DFSP tends to exhibit an indolent growth pattern, and in many cases, its symptoms are long lasting. Lindner et al., 4 who examined a series of 35 patients with DFSP who were treated at the University of Florida (Gainesville, FL), reported that symptoms ranged in duration from 6 months to 30 years (mean, 6.4 years). DFSP typically arises on the trunk of the body and is more common in men than in women. The following site distribution was observed in a series of 853 patients reported on by Enzinger and Weiss 5 : trunk, 47%; lower extremity, 20%; upper extremity, 18%; and head and neck, 14%. Rutgers et al. 6 reported a male-to-female ratio of approximately 3:2 (males, 57%; females, 43%) in a series of 264 patients with DFSP. DFSP usually 2503 © 2004 American Cancer Society DOI 10.1002/cncr.20678 Published online 22 October 2004 in Wiley InterScience (www.interscience.wiley.com).
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