A case of congenital dermatofibrosarcoma protuberans in a 2-year-old child

Dermatofibrosarcoma protuberans is a rare cutaneous soft tissue tumor with a limited potential for metastasis, but a high risk of invasive growth and local recurrence. It is histologically characterized by a dense proliferation of uniform monomorphic spindle cells in the dermis, often infiltrating subcutaneous tissues (fat, muscle, and even bone). Fibroblasts appear arranged in disorganized fascicles with a typical storiform pattern within a slightly myxoid stroma. Tumor cells of dermatofibrosarcoma protuberans usually are positive for CD34 and negative for protein S100 and factor XIIIa.