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Primary Angiosarcoma of Breast: Surgery Alone Versus Chemotherapy and/or Radiotherapy-A Systematic Review and
Konstantinos Skarentzos1, Anastasia Kourtesa2, Abraham Pouliakis1
1Second Department of Pathology, Medical School, National and Kapodistrian University of Athens, Attikon University Hospital, 12462 Athens, Greece.
Background:
Primary angiosarcoma of the breast (PAB) is a rare malignancy with no standardized treatment protocol.
Objective:
To quantify the impact of tumor grade on overall survival (OS) and evaluate the association of adjuvant chemotherapy and radiotherapy with survival in primary angiosarcoma of the breast (PAB).
Methods:
We systematically searched PubMed, Scopus, and Cochrane Library until 27 June 2025. Two reviewers independently screened studies, extracted data, and assessed risk of bias using the Newcastle-Ottawa Scale. Kaplan-Meier curve digitization was used to reconstruct individual patient data. Random-effects meta-analysis was performed for grade comparisons and therapy associations.
Results:
Eleven studies (436 patients) were included. Meta-analysis of six studies showed increasing tumor grade significantly predicted mortality, with homogeneous hazard ratios (HRs) of 1.2 (Grade 2 vs. 1) and 3.7 (Grade 3 vs. 1). Analysis of five studies revealed that adjuvant chemotherapy was associated with significantly improved survival (HR = 0.11, 95% CI: 0.02-0.45), while radiotherapy showed no benefit (p = 0.96). Included studies demonstrated low-moderate risk of bias.
Conclusions:
This first quantitative synthesis establishes histologic grade as a paramount prognostic factor in PAB and shows a strong association between adjuvant chemotherapy and survival benefit. These findings provide crucial evidence for risk stratification and support considering chemotherapy in multimodal treatment for this rare disease.
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