A Case of Myxoid Dermatofibrosarcoma Protuberans

Department of Dermatology, School of Medicine, Ewha Womans University, Seoul, Korea.
Annals of Dermatology (Impact Factor: 1.39). 08/2011; 23(3):379-81. DOI: 10.5021/ad.2011.23.3.379
Source: PubMed


Dermatofibrosarcoma protuberans (DFSP) is a slowly growing dermal spindle cell tumor and its myxoid variant, a rare type of DFSP, is characterized by extensive myxoid degeneration. We present the case of a 69-year-old woman with a multinodular reddish plaque on her trunk. Histopathologically, the tumor was located in the dermis and consisted of uniform spindle-shaped cells, showing strongly positive reaction for CD34, and negative for both S-100 and desmin. In addition to the typical storiform pattern, prominent myxoid stromal changes were demonstrated. Herein, we report an interesting case of myxoid DFSP, rarely reported in the dermatology literature.

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    ABSTRACT: We present a typical case of dermatofibrosarcoma protuberans with local recurrence 2 months after surgery and, motivated by this patient, make a review of the most important aspects. This is a rare tumor and we call special attention to the fact that its recurrence is extremely frequent, so there is absolute need to observe these patients periodically after surgery.
    Case Reports in Dermatology 05/2014; 6(2):134-9. DOI:10.1159/000362900
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    ABSTRACT: Dermatofibrosarcoma protuberans with fibrosarcomatous differentiation (DFSP-FS) is a rare variant of DFSP with a more aggressive clinical course, characterized by higher rates of local recurrence, metastasis, and death. We conducted a clinicopathologic review of all DFSP-FS cases that occurred in Alberta, Canada, from 1997 to 2007. Of the 75 DFSP cases reviewed, 4 demonstrated fibrosarcomatous differentiation. Three patients were female and one was male, and the age range was 25 to 76 years. Three tumors invaded to skeletal muscle, whereas one invaded to subcutaneous tissue only. Although perineural invasion was noted in all four cases, none exhibited lymphovascular space invasion. One local recurrence developed, and two of four tumors metastasized. Metastasis was associated with tumor size, tumor necrosis, grenz zone involvement, ulceration, thickness, and tumor grade. One patient died within 5 years of diagnosis. DFSP-FS represents a more aggressive subtype of DFSP. Several features of DFSP-FS may impart a higher risk of metastasis. © 2014 Canadian Dermatology Association.
    Journal of Cutaneous Medicine and Surgery 08/2014; 18:1-7. DOI:10.2310/7750.2014.13192 · 0.71 Impact Factor


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