Angiosarcoma in a soft tissue sarcoma cohort: real-world patterns and outcomes

Introduction Angiosarcoma is a rare, aggressive vascular sarcoma with heterogeneous clinical presentation and limited real-world outcome data. Methods We retrospectively reviewed adult soft tissue sarcoma cases managed at Dokuz Eylül University Hospital (January 2019–September 2025) and identified patients with pathologically confirmed angiosarcoma. Clinical, pathological, treatment, and survival data were extracted from electronic medical records and summarized descriptively using Kaplan–Meier methods for time-to-event outcomes. Results Among 548 soft tissue sarcoma cases, 12 patients (2.2%) had angiosarcoma; 5 (41.7%) were cutaneous and 7 (58.3%) visceral. Median age was 62 years (range, 19–88), with older age in cutaneous versus visceral disease (median 69 vs 44 years). Metastatic/unresectable disease was present in 9 patients (75.0%) at diagnosis. In the advanced setting (n=9), first-line therapy was paclitaxel-based in 6 (66.7%) and anthracycline-based in 3 (33.3%), achieving disease control in 6/9 (66.7%). Median follow-up was 28.5 months with 7 deaths. Median overall survival (OS) was 9.5 months (95% CI, 6.77–12.22) and median first-line progression-free survival (PFS) was 4.8 months (95% CI, 4.24–5.42). Conclusion Angiosarcoma was rare within an institutional sarcoma population but frequently presented at an advanced stage, with poor OS and short PFS despite contemporary therapy. Larger multi-center registries are warranted to refine risk stratification and optimize treatment sequencing.