Abstract
BACKGROUND: Limited information is available regarding survival outcomes and risk factors of dermatofibrosarcoma protuberans (DFSP). OBJECTIVE: To investigate the clinicopathologic characteristics and survival outcomes of DFSP. MATERIALS AND METHODS: The study cohort (7,567 patients) was selected from the Surveillance, Epidemiology, and End Results Program (2000-2018). Demographic and clinicopathologic variables, survival outcomes, and prognostic factors were analyzed. RESULTS: A total of 5,640 (74.53%) and 1,927 (25.47%) tumors were located in the skin and soft tissue, respectively. The median follow-up duration was 92 months. Median follow-up times were similar between patients with lymph node (107 months) and distant (102 months) metastases; the median survival time of the 89 patients (1.18%) who died of DFSP was significantly short (41 months, p < .001). Independent risk factors for cancer-specific mortality included age at diagnosis, histologic grade, and tumor size. Patients with tumors ≥10 cm in size or histologic grade III had significantly higher DFSP-specific mortality (7.07% and 10.08%, respectively, p < .001). Tumor locations and surgical procedures did not significantly influence survival. CONCLUSION: Dermatofibrosarcoma protuberans has a favorable survival prognosis, even in patients with node-positive or distant metastases. Dermatofibrosarcoma protuberans-specific mortality is significantly higher in patients with grade III or large (≥10 cm) tumors.