Chen Hao, Zhou Lu, Xiong Jingshu, Jiang Yiqun, Xu Xiulian, Zhang Wei, Zeng Xuesi, Sun Jianfang
Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China.
Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China; Email:
Zhonghua Yi Xue Za Zhi. 2015 Nov 10;95(42):3445-8.
To improve understanding of the clinical and histopathological features of early stage dermatofibrosarcoma protuberans(DFSP).
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.
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 cells 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.
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.
提高对早期隆突性皮肤纤维肉瘤（DFSP）临床及组织病理学特征的认识。
回顾性分析2008年至2014年在中国医学科学院皮肤病研究所确诊的14例早期DFSP患者的临床资料及组织病理学表现。
患者多为年轻人，平均发病年龄21岁，病程进展缓慢。初发主要表现为边界清楚的萎缩性红斑或硬化性斑块，在此基础上逐渐出现皮下结节。组织病理学显示，真皮浅层有少量肿瘤细胞，与表皮平行排列呈波浪状，而真皮深层及皮下脂肪内肿瘤细胞呈现典型的DFSP改变。肿瘤细胞表达波形蛋白和CD34，但不表达S100和CD68。
由于早期DFSP的临床及组织学特征不典型，易误诊为多种良性疾病。反复活检并结合临床特征与病理表现有助于诊断。
