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.