Clinicopathological Factors Associated with Overall Survival in Primary Cardiac Sarcoma: A Population-Based SEER Analysis

AIM: Primary cardiac sarcoma is a rare malignancy with heterogeneous clinicopathological features and limited population-level evidence. This study evaluated factors associated with overall survival (OS) in adults with primary cardiac sarcoma.
MATRERIAL and METHOD: This retrospective SEER study identified 463 adults (≥18 years) diagnosed with histologically confirmed primary cardiac sarcoma during 2000–2023 using ICD-O-3 site code C38.0 (heart) and malignant mesenchymal morphology codes. Exclusions included zero-month or missing survival, cardiac sarcoma not recorded as the first or only primary malignancy, and unresolved missing tumor size or stage. The final cohort included 268 patients. Demographic, clinicopathological, and documented treatment variables were assessed for association with OS.
RESULTS: The mean age was 47.5 ± 16.4 years, and 52.2% of patients were female. Angiosarcoma accounted for 46.6% of cases, and 36.6% had distant disease. During observation, 224 patients (83.6%) died. Median OS was 15 months (95% confidence interval [CI], 14–17); 1-, 3-, and 5-year OS estimates were 60.4%, 23.0%, and 11.6%, respectively. In the multivariable model, older age (hazard ratio [HR] per year, 1.02; 95% CI, 1.01–1.03; p<0.001) and distant versus localized disease (HR, 1.49; 95% CI, 1.01–2.22; p=0.047) were associated with higher mortality. Synovial sarcoma versus angiosarcoma (HR, 0.38; 95% CI, 0.18–0.82; p=0.014), recorded surgery (HR, 0.58; 95% CI, 0.40–0.84; p=0.004), and documented chemotherapy (HR, 0.62; 95% CI, 0.46–0.85; p=0.003) were associated with lower mortality. Radiotherapy was not significantly associated with mortality after adjustment.
CONCLUSION: Primary cardiac sarcoma was associated with poor long-term survival. Older age and distant disease were associated with higher mortality, whereas synovial histology, recorded surgery, and documented chemotherapy were associated with lower mortality. These associations warrant confirmation in larger cohorts with more detailed clinical data.