Demographics and factors influencing mortality among cardiac angiosarcoma patients and genomic studies correlation for future personalized management

Background Cardiac angiosarcoma is a rare, highly aggressive malignancy arising from endothelial cells of the heart and accounts for ∼30% of primary cardiac tumors. Understanding its epidemiology and factors influencing mortality is critical, and emerging genomic data may inform personalized management.Methods The Surveillance, Epidemiology, and End Results database was utilized to gather data from 2000 to 2021.Results A total of 194 patients were identified (102 males and 92 females). Most patients were ≤50 years (59%), with a slightly higher incidence among Black patients (18%). Overall mortality was high, with 91% of patients dying and a mean survival of 15 months. The 1-year overall survival rate was 0.461 (95% CI: 0.39–0.53). Larger tumors (>10 cm) and higher tumor grade were associated with increased mortality. Multivariate analysis showed that age 51–70 years (hazard ratio [HR] 0.57; P = 0.049), distant stage (HR 0.91; P = 0.047), absence of radiation therapy (HR 2.69; P = 0.042), and lack of surgical resection (HR 1.23; P < 0.001) were associated with higher mortality. Genomic studies have identified recurrent alterations including KDR, FLT4, PLCG1, and POT1, although most identified alterations are not yet clinically actionable.Conclusions Cardiac angiosarcoma carries a high mortality rate. Advanced age, distant disease, and lack of surgery or radiation therapy were associated with increased mortality.