Background and Aims: Cardiac angiosarcoma is a rare and highly aggressive cancer originating from the endothelial cells lining the heart. It accounts for approximately 30% of all primary cardiac tumors. Given its aggressive nature and poor prognosis, it is critical to enhance our understanding of its epidemiology and the factors influencing mortality. Methods: The Surveillance, Epidemiology, and End Results (SEER) database was utilized to gather data spanning from 2000 to 2021 using the International Classification of Diseases for Oncology (ICD-O-3), anatomical codes (C38.0-Heart), and histological code 9120. Results: We identified 194 patients with cardiac angiosarcoma, of which 102 were males and 92 were females. The majority of patients were aged 50 years or younger (59%). Non-Hispanic whites constituted the largest group (56%), followed by non-Hispanic blacks (18%), and Hispanics (16%). Mortality data showed that 91% of the diagnosed patients died (n=176), with a mean survival period of 15 months after diagnosis. The overall survival rate at 1 year was 0.461 (95% CI: 0.39-0.53), at 3 years was 0.09 (95% CI: 0.05-0.14) and at 5 years was 0.052 (95% CI: 0.03-0.10). Advanced age (51-70 years) compared to the 0-50 year age group (HR: 0.57; 95% CI: 0.003-1.14; p=0.049), distant stage (HR: 0.91; 95% CI: 0.01-1.83; p=0.047), patients who did not receive therapeutic radiation therapy compared to those who did (HR: 2.69; 95% CI: 0.10-5.28; p=0.042), and patients who did not undergo surgical resection for angiosarcoma compared to those who did (HR=1.232; 95% CI: 0.69-1,77; p<0.001) were factors associated with increased mortality. Female gender (p=0.248, as compared to males), different races of non-Hispanic White (p=0.849), non-Hispanic Black (p=0.34), non-Hispanic Asian or Pacific Islander (p=0.24), as compared to Hispanics, tumor sizes of 6-10 cms (p=0.957), or more than 10 cm (p=0.273), as compared to < 5 cm, grade of tumor (III; p=0.682, and grade IV; p=0.281, as compared to grade I), median household income of more than 65000 USD (p=0.069) as compared to less than that, and no chemotherapy (p=0.851) compared to those that received chemotherapy, were not associated with a significant increase in mortality. Conclusions: Cardiac angiosarcoma is associated with a high mortality rate. Factors contributing to increased mortality include advanced age, distant stage of disease at diagnosis, not receiving radiation therapy, and not undergoing surgical resection.
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