隆突性皮肤纤维肉瘤17例临床病理分析

Clinicopathologic analysis of 17 patients with dermatofibrosarcoma protuberans

目的探讨隆突性皮肤纤维肉瘤(DFSP)的临床病理特征、诊断及鉴别诊断要点。方法回顾性分析17例DFSP临床特征、病理组织学特点、免疫组化特点及治疗和预后。结果 17例DFSP中,男性13例,女性4例,平均年龄37.3岁。躯干部14例,头部2例,前臂1例。病变均位于真皮及皮下组织,瘤细胞呈特征性的席纹状或车辐状排列伴浸润性生长,免疫组化显示CD34和Vimentin弥散阳性,2例SMA灶区阳性,S-100均阴性。结论 DFSP是低度恶性软组织肿瘤,通过组织学及免疫组化可确诊,手术切除是主要的治疗方法。

Objective To explore the clinicopathologic characteristics,diagnosis and differential diagnosis of dermatofibrosarcoma protuberans（DFSP）. Methods The clinical features,histopathological features,immunohistochemical features,treatment and prognosis of17 cases of DFSP were retrospectively analyzed. Results Among 17 cases of DFSP,13 cases were male and 4 cases were female with an average age of 37. 3 years;there were 14 cases in the trunk,2 cases on head and 1 case in the forearm. DFSP lesions were located in the dermis and subcutaneous tissue,and the tumor cells showed characteristic mat-like or car-like arrangement with invasive growth. Immunohistochemistry showed that the expressions of CD34 and Vimentin were diffusely positive,SMA focal areas of 2 cases were positive and S-100 was negative. Conclusions DFSP is a low grade malignant soft tissue tumor. It can be diagnosed by histology and immunohistochemistry. Surgical resection is the main treatment method for DFSP.