Clinicopathological features and prognostic insights of cutaneous versus non-cutaneous angiosarcoma: A retrospective single-center cohort study.

INTRODUCTION

Angiosarcoma is a rare, aggressive malignancy of endothelial origin, accounting for 1-2 % of soft tissue sarcomas. It occurs in cutaneous (C-AS) and non-cutaneous (NC-AS) forms, with distinct clinical behaviors and prognoses. While C-AS primarily affects the head and neck, NC-AS involves visceral organs such as the heart, liver, and breast. Despite advances in treatment, survival remains poor, and recurrence is common.

METHODS

This retrospective, single-center cohort study analyzed 30 patients diagnosed with angiosarcoma at the University Hospital Zurich (2000-2023). The study compared clinicopathological characteristics, survival outcomes, and prognostic factors influencing survival and recurrence. Patients were stratified into C-AS (n = 10) and NC-AS (n = 20) groups. Overall survival (OS) and recurrence-free survival (RFS) were assessed using Kaplan-Meier and Cox regression analyses.

RESULTS

Both subtypes shared a poor prognosis, with an overall 5-year survival rate of 12 %. NC-AS had a higher early mortality rate, with a significantly worse median OS (9 vs. 36 months, p = 0.04), while early recurrence after treatment was more frequent in C-AS. High-grade tumor differentiation and metastases at diagnosis were independent predictors of poorer OS (p = 0.01; p = 0.04). Multimodal treatment, including surgery and radiotherapy, was associated with improved survival (p < 0.05). No clinicopathological factor showed a statistically significant association with RFS.

CONCLUSION

C-AS and NC-AS exhibit distinct prognostic profiles, with NC-AS associated with a higher metastatic burden and worse outcomes. Prognostic factors such as tumor grade and metastases at diagnosis influence survival. Multimodal treatment strategies appear beneficial, though recurrence remains a major challenge. Further prospective studies are needed to refine therapeutic approaches and explore emerging targeted therapies.