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 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.
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.