Background: Dermatofibrosarcoma protuberans (DFSP) is a rare type of cutaneous soft tissue sarcoma. The tumour metastasises rarely, but the percentage of recurrences is high and the tumour can be locally very aggressive.
Case description: A 50-year-old man was referred to our clinic for a long existing scar on his back, which had become painful in the last few weeks. In the hospital we decided to take a biopsy. Based on the histopathological investigation, the diagnosis DFSP was made. The patient was referred to a tertiary centre specialising in rare skin malignancies.
Conclusion: DFSP is clinically heterogeneous with the consequence that several differential diagnoses can be made. Histopathological examination is the gold standard diagnostic approach when there is a possible suspicion of DFSP. Because of the aggressiveness and the high recurrence rate it is very important to make a timely diagnosis, to allow appropriate treatment - preferably radical excision - to take place.