早期隆突性皮肤纤维肉瘤14例临床病理分析

Clinicopathological analysis of 14 cases of early stage dermatofibrosarcoma protuberans

目的 提高对早期隆突性皮肤纤维肉瘤临床及组织病理的认识.方法 回顾性分析中国医学科学院皮肤病医院2008-2014年诊断的14例早期隆突性皮肤纤维肉瘤的临床资料和组织病理特点.结果 本组患者以年轻人多见,平均发病年龄为21岁,进展较慢,临床最初表现为界限清楚的萎缩性斑或硬化性斑块,其后逐渐出现结节性皮损.组织病理显示肿瘤细胞在真皮浅层数目较少,常平行于表皮,呈波浪状排列,而在真皮下部和皮下脂肪,肿瘤表现出经典型隆突性皮肤纤维肉瘤的改变.肿瘤细胞表达波形蛋白（vimentin）和CD34,但不表达S100和CD68.结论 早期隆突性皮肤纤维肉瘤临床及组织学容易误诊多种良性疾病,重复活检及临床联系病理有助于本病的诊断.

Objective To improve understanding of the clinical and histopathological features of early stage dermatofibrosarcoma protuberans （DFSP）.Methods The clinical data and histopathological findings of 14 cases of early stage DFSP diagnosed in Institute of Dermatology,Chinese Academy of Medical Sciences from 2008 to 2014 were retrospectively analyzed.Results The patients were mostly young people.The average age of onset was 21 years old.The progress was slow.The initial main manifestations were well-circumscribed atrophic erythema or sclerotic plaque,and subcutaneous nodules appeared gradually based on the erythema.Histopathology showed a small number of tumor ceils in the upper dermis,aligned parallel to the epidermis with wavy arrangement,while the tumor cells exhibited typical DFSP changes in the lower dermis and the subcutaneous fat.The tumor cells expressed vimentin and CD34,but not S100 and CD68.Conclusions Because the clinical and histological features are atypical,the early stage of DFSP is easily misdiagnosed as various benign diseases.Repeated biopsy and combining clinical features with pathological findings are helpful for diagnosis.