Pigmented dermatofibrosarcoma protuberans の2例

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Two cases of pigmented dermatofibrosarcoma protuberans (pigmented DFSP) were reported. The first case was a 15-year-old male with a recurrent small subcutaneous tumor on the right upper arm. The original tumor, which was measured 3cm in diameter, bluish colored and elastic hard, had occurred at the age of eight. It was removed marginally and diagnosed as "blue nevus" by histological examinations; it recurred on the surgical scar after several months. Then, it was further excised with a wider surgical margin including fascia ; however, recurred again on the scar of skin grafting three years after the operation. The histological examinations showed a uniform proliferation of spindle-shaped fibroblast-like cells arranged in a storiform pattern, and interspersed melanin-containing spindle cells. The original tumor was histologically reevaluated and diagnosed as pigmented DFSP. The second case was a 30-year-old female with a slowly growing subcutaneous tumor on the right upper arm. It was multinodular, 2.5cm in diameter, reddish and partly bluish in color, and elastic hard. The histological findings were similar to those of the first case. Each case was treated by surgical excision with an appropriate surgical margin. The fibroblast-like tumor cells of both cases were positive for CD34 antigen and negative for S-100 protein on immunohistochemical examinations. Electron microscopic studies of the second case revealed that the pigmented cells had numerous mature melanosomes in their cytoplasm and were surrounded by an extracellular sheath or a basal lamina, indicating that those cells were dermal melanocytes. On the other hand, the spindle-shaped tumor cells showed a fibroblast-like appearance and no evidence of neuroectodermal differentiation.
日本皮膚悪性腫瘍学会の論文

Pigmented dermatofibrosarcoma protuberans の2例

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