Introduction: Primary Cardiac Sarcomas (PCS) are exceptionally rare malignancies, representing approximately 25% of all malignant primary cardiac tumors. Due to the rarity of these neoplasms, literature on the characteristics, optimal management, and survival outcomes in these patients is limited.
Methods: The National Cancer Database (NCDB) for soft tissue tumors was utilized to identify 826 adult patients diagnosed with tumors localized to the heart or pericardium from 2004 to 2016. Demographic information was obtained and Kaplan-Meier analysis was used to analyze overall survival of PCS. Bivariate analysis was performed with Cox proportional hazards regression models to obtain hazard ratios and assess the association of patient characteristics and treatment methods with survival.
Results: The majority of PCS patients were male (51.5%) and white (79.4%), with a mean age at diagnosis of 53 years. 41.2% were blood vessel tumors and 27.7% were sarcomas. Leiomyosarcoma and epithelial neoplasms each represented 5.2% of tumors, followed by synovial sarcomas (4.2%) and rhabdomyosarcomas (2.8%). The majority of patients were diagnosed with metastatic disease (43.2%) and received treatment (85.2%), most often with surgical resection (58.1%) or chemotherapy (57.2%).
Median overall survival was 10.9 months (95% CI: 9.6 – 12.1 months), with a cumulative survival at 1-year, 5-years, and 10-years of 27%, 10%, and 4%, respectively. Factors associated with signi cantly increased mortality (P<0.05), include increased age (HR 1.017), increased Charleson-Deyo comorbidity score (HR 1.284), and elevated stage and grade at diagnosis. Compared to blood vessel tumors, leiomyosarcoma (HR 0.696), fibroblastic (HR 0.579), osseous (HR 0.537), and fibrohystocytic (HR 0.485) histologies were associated with improved survival (P<0.05). Factors associated with signi cantly improved survival (P<0.05) included treatment by surgical resection (HR 0.500), radiation (HR 0.808), and chemotherapy (HR 0.738).
Conclusion: This is the largest study of PCS to date, and the first to analyze the NCDB. The majority of these neoplasms are blood vessel tumors and are often diagnosed at advanced stage and grade. Prognosis is poor, and all treatment modalities are associated with improved survival. Understanding of patient characteristics and overall survival is important in enhancing patient outcomes for this rare diagnosis.
