Giant dermatofibrosarcoma protuberans head and neck: a diagnostic dilemma

Dermatofibrosarcoma protuberans (DFSP) is rare, generally slow growing tumor, originating from dermal stem cell or undifferentiated mesenchymal cell. DFSP primarily occurs on the trunk and proximal extremities, with reported only 10-15% occurring in the head and neck region. There can be varied presentations of DFSP in atypical sites mimicking a benign appearance and history due to which diagnostic dilemmas can occur. The underlying malignancy needs to be unveiled by histopathology and immunohistochemistry which reveals bland spindle cells in sub epithelium arranged in whorled storiform pattern which was CD34+, suggestive of dermatofibrosarcoma protuberans. Surgical excision is the treatment of choice, wherever possible. We herein report two intriguing cases of DFSP in uncommon sites.