Dermatofibrosarcoma protuberance of the oral cavity: does it exist?

Dermatofibrosarcoma protuberance (DFSP) is a low grade spindle cell malignant tumour that is locally aggressive especially it incompletely excised. A 64-year-old man presented with intra-oral buccal mass of 34 year duration with accelerated increase in size in the last two years. CT scan showed well-circumscribed tumour with no relation to the overlying skin. Fine needle aspiration cytology revealed a highly cellular mitotically active spindle cell neoplasm with recommendation of excision with safety margins. Histological examination of the excised mass showed typical dermatofibrosarcoma protuberance with cytoplasmic positivity for Vimentin and CD34. Intra-oral sarcomas are rare and to the best of our knowledge only a single case of DFSP has been described in literature. We present another case for its unusual site, presentation, duration and surgical approach.