Unusual features of primary dermatofibrosarcoma protuberans and its myxoid recurrence

A case of Dermatofibrosarcoma Protuberans (DFSP) with unusual clinical and histological features is presented. The lesion recurred one year after surgical removal and showed myxoid degeneration of the entire lesion. Only a few cases with myxoid degeneration of the entire lesion are described in the literature. We found CD 34 stain, in combination with a panel of antibodies including fXIIIa, S100 and actin, useful in the diagnosis of this type of tumor in order to differentiate it from other neural, fibrous or muscular neoplasms.