隆突性皮肤纤维肉瘤一例

患者女，52岁，左下腹皮肤红色结节、斑块10年，皮疹增多半年。体检：患者左下腹部皮肤凹凸不平，表面可见数个黄豆到指头大小的红色丘疹及结节，表面光滑，质软如橡皮样；皮下可触及多个大小不一的结节及肿块，质地中等，表面皮肤光滑呈黄红色。皮损组织病理：真皮全层和皮下组织可见大量梭形肿瘤细胞呈席纹状排列，累及皮肤附属器，部分肿瘤细胞可见少数核分裂象。免疫组化显示，肿瘤细胞CD34、波形蛋白及BCL-2阳性，NSE （-）, Syn（-）、 S-100（-）、 SMA（-）及ⅩⅢa阴性。根据临床、组织病理及免疫组化表现诊断为隆突性皮肤纤维肉瘤。采用肿物扩大根治切除术 ＋ 植皮术，疗效满意，随访5个月未复发。

A case of dermatofibrosarcoma protuberans

A 52-year-old female patient presented with erythematous nodules and plaques for 10 years in the left lower abdomen, which had been aggravated in the past half year. Physical examination revealed several soy bean- to finger-sized, smooth, soft, rubber like, erythematous papules and nodules, and a few irregularly sized, moderately indurated subcutaneous nodules and masses with a yellowish red smooth surface in the left lower abdomen, giving a rough appearance. Skin biopsy revealed a large number of spindle tumor cells arranged in a storiform pattern throughout the full-thickness dermis and subcutaneous tissue with the involvement of skin appendages. A few mitotic figures were observed in some tumor cells. Immunohisto-chemistry of the tumor cells showed positive staining for CD34, vimentin and BCL-2, but negative staining for neuron specific enolase （NSE）, synaptophysin （Syn）, soluble protein 100 （S-100）, smooth muscle actin （SMA） and ⅩⅢa. A diagnosis of dermatofibrosarcoma protuberans was made according to the clinical, histopathologic and immunohistochemical manifestations. The patient received extended radical excision followed by skin grafting and achieved a satisfactory outcome. No recurrence was observed during a 5-month follow up.