隆突性皮肤纤维肉瘤72例临床病理学观察

目的 观察隆突性皮肤纤维肉瘤(DFSP)的临床病理特点，探讨诊断、鉴别诊断及其组织起源。方法 对72例DFSP病例进行临床表现、组织形态学、免疫组化研究，16例真皮纤维瘤(DF)、19例神经纤维瘤(NF)、17例纤维肉瘤(FS)分别作为对照。结果 72例DFSP好发中青年男性，肿瘤为单发或多发性结节，位于真皮，可浸润皮下脂肪及横纹肌。组织形态除经典的车辐状或席纹状结构外，还存在一些变异如黏液变性、伴FS区域、Bednar瘤等。DFSP组87％CD34阳性，NF组42％CD34阳性。结论 掌握DFSP的临床病理特点，避免与其它皮肤梭形细胞肿瘤尤其是DF、NF及黏液性肿瘤混淆。CD34阳性提示DFSP可能与神经鞘膜有关。

Dermatofibrosarcoma protuberans:a clinicopathologic study of 72 cases and review of literature

Purpose To investigate the clinicopathologic features of dermatofibrosarcoma protuberans(DFSP) and its diagnosis,differential diagnosis and histogenesis. Methods Seventy two cases of DFSP were studied with clinical and histopathological changes and immunohistochemical stainning, and dermatofibroma(DF, n =16),neurofibroma(NF, n =19)and fibrosarcoma(FS, n =17)were used as the control group. Results The majority of the patients were young or middle age men. The tumor consisted of solitary and multiple nodules in dermatosis and may involve in the subcuits fatty tissue or muscle. Microscopically,the tumor had classified storiform structure and some changes such as myxoid appearance, FS area and Bednar tumor. In this study, for CD34 about 87% DFSP and 42% NF were positive,DF and FS were negative. Conclusions It is important for us to make a diagnosis in grasping the clinicopathologic characteristics of DFSP. Differential diagnoses should be made between this tumor and other cutaneous spindle cell tumors, especially DF, NF and myxoid tumor.CD34 positivity suggests that DFSP may originate from nerve sheath.