Clinicopathologic features and treatment outcomes of dermatofibrosarcoma protuberans: a 25-year retrospective study

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma characterized by slow growth, a high local recurrence rate, and low metastatic potential.

To characterize the clinicopathological features and the treatment outcomes of patients diagnosed with DFSP.

Retrospective study of patients with a histopathological diagnosis of DFSP between 1997 and 2022.

Data from 42 patients with DFSP were included. The majority were female (69%) with a mean age of 49.1 years. The trunk (52%) and extremities (40%) were the most common tumor sites. Classic histologic pattern was observed in 90% of cases, while rare variants, including fibrosarcomatous, pigmented, and myxoid subtypes, were also identified. Wide local excision was the primary treatment (95%), achieving clear margins in 71% of patients. Local recurrence occurred in 4.8% and adjuvant radiotherapy was employed in 19% of cases. Two patients developed pulmonary metastases with disease progression despite treatment with imatinib.

Retrospective study based on medical and pathological records.

Cutaneous DFSP in this series demonstrated clinicopathologic features consistent with those reported in the literature, including a predilection for the trunk and middle-aged females. Histologically, most cases exhibited the classic storiform pattern with strong CD34 positivity. Wide local excision was the primary treatment modality, with a relatively low recurrence rate compared to the literature, possibly influenced by the use of adjuvant radiotherapy in select patients. Although uncommon, metastasis occurred in cases with recurrent disease and fibrosarcomatous transformation. Ongoing research into systemic therapies and molecularly targeted treatments is needed to improve outcomes in advanced DFSP.