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. 2021 Feb;25(2):104-110.
doi: 10.14744/AnatolJCardiol.2020.78107.

Incidence, prognostic significance, and survival outcomes of primary cardiac sarcoma: An updated population-based retrospective study

Mohammad Bakri Hammami  1 , Mohd Zaki Al-Wawi  2 , Huwaida Fazel  3 , MohamadAnas Oudih  4
Affiliations

Incidence, prognostic significance, and survival outcomes of primary cardiac sarcoma: An updated population-based retrospective study

Mohammad Bakri Hammami et al. Anatol J Cardiol. 2021 Feb.

Abstract

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.

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Conflict of interest statement

Conflict of interest: None declared.

Figures

Figure 1
Figure 1
Flow chart of cases included for the incidence analysis (408) and survival analysis (385)
Figure 2
Figure 2
Histogram of cardiac sarcoma patients’ age at diagnosis
Figure 3
Figure 3
Annual percentage change of cardiac sarcoma according to gender
Figure 4
Figure 4
Comparison of cumulative survival of patients according to (a) surgical treatment (b) stage and (c) year of diagnosis
See this image and copyright information in PMC

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