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      Prevalence of primary cardiac tumor malignancies in retrospective studies over six decades: a systematic review and meta-analysis

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          Abstract

          The incidence of patients diagnosed with primary malignant cardiac tumors (PMCTs) has increased greatly in the past few decades. Whether this rising prevalence is due to overdiagnosis or an increased malignancy rate of primary cardiac tumors (PCTs) remains unclear. Therefore, we performed a systematic review and meta-analysis of published retrospective studies to determine whether the malignancy rate has been increasing over time. Published studies containing relevant data between 1956 and 2014 were evaluated. Two authors searched for all retrospective studies that included patients diagnosed with PCT and PMCT. Two other investigators independently extracted the data, and discrepancies were resolved by consensus. A random-effects meta-analysis model and cumulative meta-analysis model were used to evaluate the pooled prevalence and trend of dynamic change in PCT malignancies. The effects of time, study period and sample size were studied using a logit-linear regression model with robust error variance and a time variable. Thirty-eight studies involving 5,586 patients were analyzed. The pooled prevalence of PMCT among the patients diagnosed with PCT was 9.9% (95% CI, 8.4% to 11.4%) (I 2=70%; P< 0.001), and this prevalence has been stable since around 2003. In the regression model, the malignancy odds ratio remained stable from 1975 onward, and no time effect was observed. Our study confirms that PMCT is uncommon, and the prevalence of PCT malignancies remained stable in the past few decades. The clinically observed increase in incidence is unlikely to reflect a true population-level increase in tumorigenesis. This result strongly suggests that the observed increase in incidence of PMCT most likely reflects increased diagnostic detection over time.

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          Primary sarcomas of the heart.

          Seventy-five primary sarcomas of the heart were classified by histologic appearance as angiosarcoma (26 cases), undifferentiated sarcoma (18 cases), osteosarcoma (9 cases), fibrosarcoma (6 cases), malignant fibrous histiocytoma (6 cases), leiomyosarcoma (4 cases), myxosarcoma (3 cases), synovial sarcoma (2 cases), and neurofibrosarcoma (1 case). The ages of the patients ranged from 1 to 75 years at the time of presentation (mean, 39 years). Angiosarcomas were predominantly right-sided and osteosarcomas left-sided. Forty patients treated surgically were examined, and survival correlated with clinical and histologic parameters. the survival rate was poor, with a mean of 11 months and median of 6 months. By univariate analysis, the survival rate was more favorable for patients with tumors located on the left side of the heart, without necrosis, with a low mitotic count, and without metastasis at diagnosis. Survival rates were better in patients receiving chemotherapy and radiation therapy. Age, gender, presence of differentiation, and histologic type did not affect prognosis. By multivariate analysis, a low level of mitotic activity and any therapy were the only significant factors affecting survival rate. Immunostaining with commercially available antisera was useful in the diagnosis of sarcoma but not in subclassification of 19 tumors so tested. Although the prognosis for patients with cardiac sarcomas is dismal, histologic grading is useful in predicting outcome, as has been shown for soft tissue sarcomas of other sites.
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            Survival after resection of primary cardiac tumors: a 48-year experience.

            Primary cardiac tumors are rare but have the potential to cause significant morbidity if not treated in an appropriate and timely manner. To date, however, there have been no studies examining survival characteristics of patients who undergo surgical resection. From 1957 to 2006, 323 consecutive patients underwent surgical resection of primary cardiac tumors; 163 (50%) with myxomas, 83 (26%) with papillary fibroelastomas, 18 (6%) with fibromas, 12 (4%) with lipomas, 28 (9%) with other benign primary cardiac tumors, and 19 (6%) with primary malignant tumors. Operative (30 day) mortality was 2% (n=6). Univariate analysis indicated that patients who underwent resection of fibromas and myxomas had superior survival characteristics in comparison to the remainder of tumor variants; these results were consistent after adjusting for age at surgery, year of surgery, and cardiovascular risk factors. Based on actuarial characteristics of the 2002 U.S. population, patients who underwent myxoma resection had survival characteristics that were not significantly different from that of an age and gender matched population (SMR 1.11, P=0.57) whereas those who underwent resection of fibromas (SMR 11.17, P=0.002), papillary fibroelastomas (SMR 3.17, P=0.0003), lipomas (SMR 5.0, P=0.0003), other benign tumors (SMR 4.63, P=0.003), and malignant tumors (SMR 101, P<0.0001) had significantly poorer survival characteristics. Furthermore, malignant tumors in younger patients were highly fatal (HR 0.899, P<0.0001). Although the most significant predictor of mortality was tumor histology, survival was also influenced the by the duration of CPB and NYHA III/IV; the impact of these risk factors varied with time. The cumulative incidence of myxoma recurrence was 13% and occurred in a younger population (42 versus 57 years, P=0.003) with the risk of recurrence decreased after 4 years. Surgical resection of primary cardiac tumors is associated with excellent long-term survival; patients with cardiac myxomas have survival characteristics that are not significantly different from that of a general population. Predictors of mortality are primarily related to tumor histology but also include clinical characteristics such as symptomatology and duration of CPB.
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              Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases.

              To investigate the characteristics and pathological features of primary cardiac tumors and to evaluate the diagnostic sensitivity of echocardiography in primary cardiac tumors, all pathologic and echocardiographic records at the Chinese PLA general hospital and its satellite hospitals between January 1st, 1990 and January 1st, 2000 were reviewed to identify patients with a confirmed diagnosis of primary cardiac tumors. A total of 149 patients who had complete echocardiographic records and who were diagnosed with primary cardiac tumors were included in the study. Pathologic and echocardiographic records were reviewed retrospectively to evaluate the presence, location and histologic type of the tumors. The majority (n=118, 79.2%) of cases had been diagnosed with benign tumors. Myxoma was the most common histologic type accounting for 50.0% of total cardiac tumors. Lipoma was the second most common type of benign tumor. Among cases with malignant tumors (n=31, 20.8%), unclassified sarcoma (n=7), angiosarcoma (n=6) and rhabdomyosarcoma (n=6) were the common histologic types of primary malignant tumor. Non-myxomatous benign tumors were more likely to have occurred in the ventricle than myxomas (17/43, 39.5% vs. 7/75, 9.3%; P=0.00). The proportion of pericardium involvement in the malignant tumors (8/31, 25.8%) was significantly higher than that in the myxomas (0/75, 0%; P=0.00) and non-myxomas (2/43, 4.7%; P=0.01). The diagnostic sensitivity of transthoracic and transesophageal echocardiography was 93.3% (139/149) and 96.8% (30/31), respectively. The study, using a relatively large sample, confirms that myxoma was the most common primary cardiac tumor. The locations of tumor involvement varied by types of tumor. Echocardiography may be a useful tool for early diagnosis of primary cardiac tumors.
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                Author and article information

                Journal
                Oncotarget
                Oncotarget
                Oncotarget
                ImpactJ
                Oncotarget
                Impact Journals LLC
                1949-2553
                27 June 2017
                24 April 2017
                : 8
                : 26
                : 43284-43294
                Affiliations
                1 Department of Thoracic and Cardiovascular Surgery, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu, China
                2 Department of Epidemiology, School of Public Health, Nanjing Medical University, Nanjing, Jiangsu, China
                Author notes
                Correspondence to: Xin Chen, stevecx1@ 123456163.com
                Article
                17378
                10.18632/oncotarget.17378
                5522145
                28489604
                4519c47c-550e-4366-9beb-6aa3ee7fc84d
                Copyright: © 2017 He et al.

                This article is distributed under the terms of the Creative Commons Attribution License (CC-BY), which permits unrestricted use and redistribution provided that the original author and source are credited.

                History
                : 30 November 2016
                : 30 March 2017
                Categories
                Meta-Analysis

                Oncology & Radiotherapy
                prevalence,malignancy,primary cardiac tumors,retrospective studies,meta-analysis

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