隆突性皮肤纤维肉瘤一例
A case of dermatofibrosarcoma protuberans
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.
更多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.
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