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: To study the clinicopathologic features and differential diagnosis of myxoid dermafibrosarcoma protuberans (DFSP). The clinical and pathologic features of 16 cases of myxoid DFSP were reviewed. There were altogether 15 males and 1 female. The age of the patients ranged from 11 to 73 years (mean = 47 years and median = 48 years). The commonest site of involvement was trunk (number = 11), followed by shoulder (number = 2), head and neck (number = 2), and extremity (number = 1). Similar to conventional DFSP, most patients presented with a slowly enlarging subcutaneous nodule which showed a rapid recent growth in some cases. Amongst the 16 cases studied, 12 occurred de novo and 4 represented local recurrence. The tumors ranged from 2 to 10 cm in greatest dimension (mean = 5 cm and median = 4 cm). Histologically, they were poorly circumscribed and located in the dermis, with focal infiltration into the underlying subcutaneous tissue. Seven cases were purely myxoid and composed of spindly and stellate cells with delicate arborizing vascular meshwork. The remaining 9 cases were predominantly myxoid (> 50%), with 5 cases containing cellular areas resembling conventional DFSP and 4 cases showing fibrosarcomatous transformation. In addition, foci of giant cell fibroblastoma-like areas were noted in 1 case. Immunohistochemical study showed that the tumors cells were positive for CD34. The staining was weak in the myxoid areas, as compared with conventional DFSP. Of the 4 recurrent cases, one patient developed lung metastases. Myxoid DFSP represents a rare variant of DFSP and may pose important diagnostic pitfalls. It is especially so if the tumor purely consists of myxoid element. Familiarity with the histologic features helps to avoid misdiagnosis.
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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.
    Full-text · Article · May 2014 · Case Reports in Dermatology
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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.
    No preview · Article · Aug 2014 · Journal of Cutaneous Medicine and Surgery
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