隆突性皮肤纤维肉瘤临床病理学分析

Clinical and pathological analysis of 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2017年-2020年诊断的12例DFSP患者的临床表现、组织病理学特征、免疫组化特点及治疗、预后。结果:12例DFSP患者中,男性9例,女性3例,腹部7例、头颈部2例、肩部1例、臀部1例,上肢1例。肿瘤组织在真皮层及皮下呈浸润性生长并形成车辐状或席纹状结构,免疫组化检测CD34、Vimentin弥漫阳性,S-100均阴性。1例FS-DFSP CD34在FS区域表达减弱,Ki-67指数较高。结论:通过组织病理学、免疫组化检测可以确诊DFSP,规范性手术切除是主要的治疗方式。

Objective:To investigate the clinicopathological features,diagnosis and differential diagnosis of fibrosarcoma protuberans.Methods:The clinical manifestations,histopathological features,immunohistochemical features,treatment and prognosis of 12 DFSP diagnosed from 2017 to 2020 were retrospectively analyzed.Results:Among the 12 cases of DFSP,there were 9 males and 3 females.There were 7 cases of abdomen,2 cases of head and neck,1 case of shoulder,1 case of buttocks,and 1 case of upper limb.The tumor tissue is infiltrating in the dermis and subcutaneously and forms"storiform"structure,Immunohistochemical CD34,Vimentin diffuse positive,S-100 negative.One case FS-DFSP CD34 decreased expression in the FS region and a higher Ki-67 index.Conclusion:DFSP can be confirmed by histopathology and immunohistochemistry,standard surgical resection is the main treatment.