Survival outcomes in patients with primary cardiac sarcoma in the United States.

Objective: We aim to evaluate the survival outcomes of primary cardiac sarcoma in a US nationwide cancer database.

Methods: The Surveillance, Epidemiology, and End Results database was queried to identify patients with primary cardiac sarcoma from 1973 to 2015. Kaplan-Meier analysis and log-rank tests were performed to compare overall survival for subpopulations, stratified on year at diagnosis (dichotomized into 2 periods: 1973-2005 and 2006-2015), pathological types, whether patients were treated with surgery or not, and surgery and chemotherapy combinations. Multivariable Cox regression was performed to estimate the adjusted hazard ratios and 95% confidence intervals of potentially clinically important factors.

Results: A total of 442 patients (mean age, 47.2 ± 18.7 years; male 52.0%) were identified. Most patients were white (78.1%) and diagnosed at age 20 to 60 years (70.2%). Angiosarcoma (43.2%) was the most common histologic type. Overall, the median survival was 7 months, and the 1-, 3-, and 5-year survivals were 40.7%, 15.6%, and 9.8%, respectively. Patients who were diagnosed within the recent decade (2006-2015) did not achieve a better overall survival (P = .13). Surgery (adjusted hazard ratio, 0.49; 95% confidence interval, 0.37-0.64; P < .001) and chemotherapy (adjusted hazard ratio, 0.70; 95% confidence interval, 0.54-0.92; P = .009) were independently associated with improved overall survival. Increasing age (adjusted hazard ratio of 5-year increment, 1.07; 95% confidence interval, 1.04-1.11; P < .001) was independently associated with worse survival.

Conclusions: At the population level, primary cardiac sarcoma has a poor prognosis. Both surgery and chemotherapy are associated with improved survival, whereas increasing age at diagnosis was associated with worse survival.