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

Angiosarcoma is a rare, aggressive vascular sarcoma with heterogeneous clinical presentation and limited real-world outcome data.

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.

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).

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.