Cardiac fibroma presenting as sudden unexpected death in an adolescent

Cronin, Benjamin; Lynch, Matthew; Parsons, Sarah

doi:10.1007/s12024-014-9582-3

Cited by 16 publications

(5 citation statements)

References 15 publications

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“…This study utilized the SEER database, which is considered a highly reliable source of epidemiologic information (incidence and prevalence), and survival assessment. However, inaccurate estimation of the prevalence of cardiac tumors in the SEER data might be present as these tumors can present by sudden cardiac death ( 45 ), hence not documented, as the SEER data reported only living cases not the postmortem diagnosis. Studying the impact of the geographic location on survival from the SEER data might be changed over time given the fact of patients’ migration to different areas.…”

Section: Discussionmentioning

confidence: 99%

Geographic variation in malignant cardiac tumors and their outcomes: SEER database analysis

et al. 2023

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IntroductionPrimary malignant cardiac tumors (PMCTs) are rare. Geographical distribution has been demonstrated to affect cancer outcomes, making the reduction of geographical inequalities a major priority for cancer control agencies. Geographic survival disparities have not been reported previously for PMCT and the aim of this study is to compare the prevalence and the long-term survival rate with respect to the geographic location of PMCTs using the Surveillance, Epidemiology, and End Results (SEER) research plus data 17 registries between 2000 and 2019.MethodsThe SEER database was queried to identify geographic variation among PMCTs. We classified the included states into 4 geographical regions (Midwest, Northeast, South and West regions) based on the U.S. Census Bureau-designated regions and divisions. Different demographic and clinical variables were analyzed and compared between the four groups. Kaplan Meier curves and Cox regression were used for survival assessment.ResultsA total of 563 patients were included in our analysis. The median age was 53 years (inter-quartile range (IQR): 38 - 68 years) and included 26, 90, 101, and 346 patients from the Midwest, Northeast, South, and West regions respectively. Sarcoma represented 65.6% of the cases, followed by hematological tumors (26.2%), while mesothelioma accounted for 2.1%. Treatment analysis showed no significant differences between different regions. Median overall survival was 11, 21, 13, and 11 months for Midwest, Northeast, South and West regions respectively and 5-year overall survival was 22.2%, 25.4%, 14.9%, and 17.6% respectively. On multivariate Cox regression, significant independent predictors of late overall mortality among the entire cohort included age (Hazard Ratio [HR] 1.028), year of diagnosis (HR 0.967), sarcoma (HR 3.36), surgery (HR 0.63) and chemotherapy (HR 0.56).ConclusionPrimary malignant cardiac tumors are rare and associated with poor prognosis. Sarcoma is the most common pathological type. Younger age, recent era diagnosis, surgical resection, and chemotherapy were the independent predictors of better survival. While univariate analysis revealed that patients in the South areas had a worse survival trend compared to other areas, geographic disparity in survival was nullified in multivariate analysis.

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Section: Discussionmentioning

confidence: 99%

Geographic variation in malignant cardiac tumors and their outcomes: SEER database analysis

et al. 2023

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“…11 In addition, SEER includes only patients diagnosed with cancer before death, not postmortem findings. Although PMCTs commonly present with dyspnea, chest pain, palpitations, and edema, [11][12][13] they can also remain clinically silent until causing ventricular arrhythmias 13 and sudden cardiac death, 14 thus escaping inclusion in SEER. Nevertheless, more in line with our findings, a recent study in Grosseto's county in Italy (1998-2011) estimated the incidence of PMCTs at ≈130 per 100 million persons.…”

Section: Discussionmentioning

confidence: 99%

Characteristics and Survival of Malignant Cardiac Tumors

et al. 2015

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Background-The aim of this study was to investigate the incidence, histopathology, demographics, and survival associated with primary malignant cardiac tumors (PMCTs). Methods and Results-We queried the Surveillance, Epidemiology and End Results (SEER) 18 registry from the National Cancer Institute for all PMCTs diagnosed from 1973 to 2011. We describe PMCT histopathology and incidence, comparing characteristics and survival of these patients with those of patients with extracardiac malignancies of similar histopathology. From a total of 7 384 580 cases of cancer registered in SEER, we identified 551 PMCTs (0.008%). The incidence of PMCT diagnosis is 34 cases per 100 million persons and has increased over time (25.1 in 1973-1989, 30.2 in 1990-1999, and 46.6 in 2000-2011). Most patients are female (54.1%) and white (78.6%) with median age at diagnosis of 50 years. The most common PMCTs are sarcomas (n=357, 64.8%), followed by lymphomas (n=150, 27%) and mesotheliomas (n=44, 8%). Most patients are diagnosed with tissue sample (96.8%). Although use of chemotherapy is not documented in SEER, 19% of patients received radiation and 44% had surgery. After a median follow-up of 80 months, 413 patients had died. The 1-, 3-, and 5-year survival rates were 46%, 22%, and 17% and have improved over the eras, with 1-, 3-, and 5-year survival rates of 32%, 17%, and 14% for 1973 to 1989 and 50%, 24%, and 19% for 2000 to 2011 (P=0.009). Cardiac sarcomas and mesotheliomas are the most lethal PMCTs, with 1-, 3-, 5-year survival rates of 47%, 16%, and 11% and of 51%, 26%, and 23% compared with 59%, 41%, and 34% for lymphomas, respectively (log rank test P<0.001). Patients with cardiac lymphomas and sarcomas are younger and have worse survival than patients with extracardiac disease of similar histopathology (P<0.001). Conclusions-PMCTs are extremely rare and continue to be associated with poor prognosis. Over the past 5 decades, the incidence and survival of patients diagnosed with PMCT appear to have increased.

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“… 92 Sudden cardiac death can be the initial presentation in 25% of patients. 93 Other presentations include heart failure, syncope, and arrhythmias. They almost always manifest as single lesions, which help to distinguish them from rhabdomyoma.…”

Section: Intracavitary and Myocardial Massesmentioning

confidence: 99%