Abstract
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.