Primary Cardiac Sarcoma: Impact of Tumor Location and Biology in Surgically Resected Cases

OBJECTIVE(S): We evaluated perioperative and long-term outcomes after surgical resection of primary cardiac sarcoma (PCS) according to anatomical tumor location and identified predictors of survival. METHODS: We retrospectively reviewed 158 patients undergoing 172 PCS resections between 1998 and 2026. Patients were stratified according to tumor location as left heart (n=67), right heart (n=58), or great vessel sarcomas (n=33). Survival was evaluated using Kaplan-Meier and multivariable Cox analysis, including sensitivity analysis excluding operative mortality. RESULTS: Operative mortality was 13.4%. Median post-resection survival was 1.3 years; 1-, 3-, and 5-year survival was 59.1%, 27.7%, and 16.9%, respectively. Location was associated with survival (p=0.037), driven by lower mortality for great vessel versus left heart sarcomas (HR 0.50; 95% CI 0.29-0.86). Metastatic disease/direct pulmonary invasion (HR 1.69, 95% CI 1.15-2.50), age (HR 1.02/year; 95% CI 1.00-1.03) and R2 resection (HR 5.11; 95% CI 2.60-10.05) were independently associated with poorer survival; R1 resection was not. Excluding operative mortality, location was no longer significant (p=0.23), whereas histology was significant overall (p=0.012); UPS/intimal/spindle-cell sarcoma (HR 0.48; 95% CI 0.26-0.90) and leiomyosarcoma (HR 0.26; 95% CI 0.12-0.60) were associated with improved survival versus angiosarcoma. Metastatic disease/direct pulmonary invasion and R2 resection remained significant. CONCLUSIONS: Tumor location influenced operative management and was associated with post-resection survival, but this was attenuated after excluding operative deaths. Among operative survivors, long-term outcomes were driven predominantly by histology, extracardiac disease, and gross residual disease. Although surgical resection remains a cornerstone of treatment, persistently poor survival highlights the need for effective biology-driven systemic therapies.