隆突性皮肤纤维肉瘤19例临床病理分析及文献复习

Dermatofibrosarcoma protuberans: a clinicopathologic study of 19 cases and review of literature

目的探讨隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)的临床病理特征、诊断、鉴别诊断、免疫组化及预后。方法对19例隆突性皮肤纤维肉瘤患者的临床、病理资料进行分析总结,并复习相关文献。结果 DFSP的病变位于真皮,可侵及皮下脂肪组织及横纹肌,组织学表现为梭形细胞呈典型的席纹状或车辐状排列,并存在多种变异型;免疫组化CD34和vimentin弥漫强阳性,S-100、SMA阴性。结论 DFSP形态变化多样,需与其它皮肤梭形细胞肿瘤相鉴别,仔细寻找DFSP组织学经典区域,并借助免疫组化明确诊断。

Objective To investigate the effect of dermatofibrosarcoma protuberans（ DFSP） of the clinical and pathological features, diagnosis, differential diagnosis, immunohistochemistry and prognosis.Methods 19 cases of dermatofibrosarcoma protuberans were analyzed and summarized for morpathological features,and reviewed relevant literatures. Results DFSP lesions were located in the dermis and involved in the subcuit fatty tissue or muscle. Microscopically,the tumor had classified storiform structure and some changes.Immunohistochemically,all tumor cells were diffusely and strongly positive for CD34 and vimentin,and negative for S-100 protein,SMA. Conclusions DFSP morphology varied should be differentiated with other spindle cell tumors of the skin,carefully looking for DFSP histological classical region,and using the immunohistochemical for diagnosis.