Objective: Primary cardiac sarcoma, a rare tumor with an aggressive course and imprecise prognosis, constitutes over 95% of all malignant cardiac tumors. Given the sparsely available evidence, there is a paucity of information regarding current knowledge on cardiac sarcoma. This study aimed to determine the incidence and incidence-based rates, patient characteristics, treatment modalities, and survival factors of cardiac sarcoma.

Methods: A retrospective analysis of the incidence, incidence-based mortality rates and characteristics of cardiac sarcoma between 1975 and 2016 was carried out using the Surveillance, Epidemiology, and End Results (SEER) database. The National Cancer Institute’s Joinpoint Regression program was used to calculate the Annual Percentage Changes (APC). Univariate and multivariate regression analysis were used to determine the survival characteristics.

Results: A total 408 patients were identified for the incidence analysis, while 385 eligible patients were identified for the survival analysis. The mean age at diagnosis was 46.3±17.9 years. The incidence rate (per 100.000 per year) of cardiac sarcoma within the indicated years was 0.22, with an increased APC of 1.7 (p=0.013, 95% CI=0.5–2.9). A total of 251 (61.5%) patients underwent surgery, 93 (22.8%) patients received adjuvant radiotherapy, and 197 (50.2%) patients received chemotherapy. Surgical resection, chemotherapy, stage of tumor, and younger age significantly improved the survival outcomes (p<0.001).

Conclusion: Cardiac sarcoma is a rare type of soft tissue sarcomas with poor prognosis. Over the past 30 years, the incidence of cardiac sar-coma has been on the increase. Surgery remains the mainstay of management. Further studies are needed to compare different diagnostic and treatment modalities so as to ascertain the best treatment option that would enhance survival and prognosis of cardiac sarcoma.