Survival Outcomes and Prognostic Factors of Dermatofibrosarcoma Protuberans: A Population-Based Retrospective Cohort Analysis

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.