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. 2025 Sep:273:156139.
doi: 10.1016/j.prp.2025.156139. Epub 2025 Jul 20.

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

Davide Voci  1 , Alexandru Grigorean  2 , Julia Neuenschwander  3 , Franca Lisy  4 , Riccardo M Fumagalli  2 , Tim Sebastian  2 , Nils Kucher  2 , Rolf P Engelberger  5
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Free article

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

Davide Voci et al. Pathol Res Pract. 2025 Sep.
Free article

Abstract

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.

Keywords: Angiosarcoma; Chemotherapy; Immunotherapy; Prognostic factors; Targeted therapy.

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Conflict of interest statement

Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Rolf P. Engelberger reports a relationship with Concept Medical Inc. that includes: funding grants. Nils Kucher reports a relationship with Concept Medical Inc. that includes: funding grants and speaking and lecture fees. Nils Kucher reports a relationship with Bard Ltd that includes: funding grants. Nils Kucher reports a relationship with Bentley InnoMed GmbH that includes: funding grants. Nils Kucher reports a relationship with Boston Scientific Corporation that includes: funding grants and speaking and lecture fees. Nils Kucher reports a relationship with Inari Medical Inc that includes: funding grants and speaking and lecture fees. Nils Kucher reports a relationship with Sanofi that includes: funding grants. Nils Kucher reports a relationship with Bayer AG that includes: funding grants and speaking and lecture fees. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper

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