Dermatofibrosarcoma protuberans has a high rate of recurrence, presumably as a consequence of persistent residual tumour. This is probably the main reason why repeated surgical treatment is frequently required. In the article we present our experience of surgical treatment of such malignancy on the neck and shoulder in the elder male after ten years follow-up period. The case is interesting because we started with aggressive treatment relatively late in the unfavourable, fully-developed stage of the disease, with multiple satellite nodules spreading and signs of partial myxoid degeneration of the largest tumour mass itself. Despite initial wide excision, five local recurrences - fortunately without regional or distant metastases - were observed during the first eight years. The recurrences appeared mostly in the region of greatest tumour degeneration and infiltration and where the excision margins were less than 2 cm apart from the visible tumour mass. A sufficiently wide excision, long-term follow-up and good co-operation between surgeon and experienced pathologist are crucial for the strategy of treatment and the prognosis of this unusual malignancy.

        Klíčová slova: dermatofibrosarcoma protuberans, fibrohistiocytic tumors, neoplasm