Cutaneous Angiosarcoma Subtypes: A Quantitative Systematic Review of Demographics, Treatments, and Outcomes Within Published Patient-Level Cases

BACKGROUND: Cutaneous angiosarcoma (cAS) is a highly aggressive malignancy arising from the vascular endothelium. Given its rarity, there is insufficient data detailing patient demographics, management, and survival outcomes. OBJECTIVE: To systematically compile published patient-level cases of cAS and to quantify and analyze data on demographics, management, and outcomes while determining prognostic indicators. MATERIALS AND METHODS: Searches of EBSCOhost, MEDLINE, EMBASE, and the Cochrane Library generated 1,500 cases of cAS with individual level data available. PRISMA guidelines were followed. RESULTS: Cutaneous angiosarcoma presented most often on the scalp of elderly men. Metastasis occurred in 36.3% of cases. Aggregate 5-year survival was 31.6% with the median survival of 25 months. The best 5-year survival was in the radiation-associated subtype (48.8%), whereas the worst was in the Stewart-Treves subtype (21.6%). Using multivariate analysis, gender, age group, disease subtype, treatment modality, and metastasis at presentation had significant effects on survival outcomes ( p < .05). CONCLUSION: The breadth of information obtained enables this study to serve as a resource that clinicians may reference when they encounter cAS.