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Despite being extremely rare in children, primary benign cardiac tumors can cause malignant ventricular arrhythmias (VA) or even sudden cardiac death. To assess the predictors of cardiovascular death and malignant VAs, we designed a retrospective single-center study enrolling paediatric patients. We defined as primary outcome a composite of cardiovascular death, sustained VT, ventricular fibrillation and rapid, symptomatic non-sustained VT. Our secondary endpoint was to assess the prevalence of clinically significant arrhythmias in our population. METHODS AND RESULTS: We fitted a multivariate Cox regression model to assess the predictors of the primary outcome. Over a period of 38 years, a total of 97 children were enrolled in the study. Among them, there were 73 rhabdomyomas, 13 fibromas, 3 myxomas, 3 teratomas, 1 lipoma, 2 haemangiomas and 2 fibroelastomas. Over a median follow up of 10.53 years, 16 patients met the primary outcome. Kaplan Meier unadjusted survival estimates showed that tumor dimensions larger than 2.3 cm and diagnosis of fibroma predicted worse outcomes compared with smaller tumors or other histotypes, (log rank p < 0.0002 and < 0.0001 respectively). In multivariate Cox proportional hazards analysis, diagnosis of fibroma and tumor dimensions were independently associated to the primary endpoint (HR: 5.06, 95 %CI (1.3-19); and 1.26 • (1.05-11), respectively). Clinically significant arrhythmias were reported in 24.5 % of the study population. CONCLUSIONS: Among paediatric primary cardiac tumors, type and dimensions may predict the hazard of malignant VAs and cardiac death., Competing Interests: Declaration of competing interest The Authors declare no conflict of interest., (Copyright © 2024. Published by Elsevier B.V.)

Objective: Primary cardiac tumors are uncommon, often benign, but can be potentially life threatening. Minimally invasive endoscopic (ENDO) techniques have been shown to be a feasible alternative for tumor resection compared with conventional sternotomy (CS). This study compared the clinical and surgical outcomes of a small series of patients undergoing cardiac tumor resection operations., Methods: Between November 2009 and December 2022, 34 consecutive patients underwent cardiac tumor resection using either ENDO ( n = 21) or CS ( n = 13) techniques. We compared early perioperative outcomes, echocardiographic outcomes, and long-term clinical and tumor recurrence outcomes., Results: Baseline characteristics were similar between groups; however, the ENDO group included younger patients (56 ± 16 vs 62 ± 17 years) and more female patients (83% vs 53%). The tumor was located in the left atrium ( n = 19, 56%), right atrium ( n = 5, 15%), or either ventricle ( n = 4, 12%). In-hospital mortality and stroke frequency were similar for both groups ( n = 0). There was no significant difference in cardiopulmonary bypass or cross-clamp times, respiratory or renal failure, or intensive care unit or hospital lengths of stay. At follow-up (ENDO, 42 [2 to 131] months vs CS, 54 [1 to 156] months), there were no deaths in the ENDO group and 2 patients died in the CS group ( P = 0.21). No patients in either group experienced tumor recurrence., Conclusions: In selected patients, both ENDO and CS approaches to primary cardiac tumor resection were safe, effective, durable, and associated with similarly good early and late results., Competing Interests: Declaration of Conflicting InterestsThe authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: M.W.C. is supported as the Ray and Margaret Elliott Chair in Surgical Innovation and has received Speaker’s honoraria from Medtronic, Edwards, Terumo Aortic, and Artivion.

Background: Myxomas are primary cardiac tumours that may be detected incidentally due to embolic events, intracardiac obstructive features or non-specific structural symptoms. The aim of this study was to share our experience of clinical features, diagnostic methods, surgical procedures and postoperative follow up of surgically treated cardiac myxomas., Methods: Data of 34 patients who underwent surgery for a cardiac myxoma between January 2006 and June 2022 were retrospectively analysed. Group 1 ( n = 19) consisted of patients who were symptomatic and group 2 ( n = 15) patients were asymptomatic. The medical records of the patients, their clinical status, diagnostic methods, operation information and postoperative course data were collected and recorded., Results: A total of 34 patients (16 female; mean age 54.5 ± 8.8 years) underwent cardiac myxoma resection with cardiopulmonary bypass. Fifteen (44.1%) patients were diagnosed incidentally with asymptomatic myxoma. An additional cardiac surgical procedure was performed in six patients (17.7%). The 34 cardiac myxomas that were surgically resected were localised in the left atrium in 25 patients (73.5%) and in the right atrium in nine patients (26.5%). Patients' most common symptoms were dyspnoea (42.1%), palpitations (21.1%), ischaemic stroke (15.8%) and syncope (10.5%). There was no incident of 30-day mortality and no recurrence was observed in any patient during the follow-up period. The duration of surgical intervention in symptomatic patients was significantly shorter than in asymptomatic patients ( p = 0.0001), but there was no statistical difference in terms of characteristics., Conclusions: Myxomas are benign tumours, but they are serious pathologies that require early treatment because of signs of obstruction, embolic complications and confusion, with left atrial thrombus in the differential diagnosis.

Background: The survival outcome for primary cardiac malignant tumors (PMCTs) based on race has yet to be fully elucidated in previously published literature. This study aimed to address the general long-term outcome and survival rate differences in PMCTs among African Americans and Caucasian populations., Methods: The 18 cancer registries database from the Surveillance, Epidemiology, and End Results (SEER) Program from 1975 to 2016 were utilized. Ninety-four African American (AA) and 647 Caucasian (CAU) patients from the SEER registry were available for survival analysis. The log-rank test was used to compare the difference in mortality between two populations and presented by the Kaplan-Meier curves. A multivariate Cox proportional hazards regression was used to determine the independent predictors of all-cause mortality., Results: The overall 30-day, 1-year, and 5-year survival rates were 74%, 44.3%, and 16.6%, respectively, with a median survival of 10 months. There was no significant difference in survival rate between the two races (p-value = 0.55). The 1-year survival rate improved significantly during the study timeline in the AA population (13.3% during 1975-1998, 40.9% during 1999-2004, 50% during 2005-2010, and 59.7% during 2011-2016, p-value = 0.0064). Age of diagnosis, type of tumor, disease stage, and chemotherapy administration are the main factors that predict survival outcomes of PMCT patients. Interactive nomogram was developed based on significant predictors., Conclusions: PMCTs have remained one of the most lethal diseases with poor survival outcome. Survival rate improved during the timeline in AA patients, but in general, racial differences in survival outcome were not observed., (© 2021 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Objective: We aim to evaluate the survival outcomes of primary cardiac sarcoma in a US nationwide cancer database., Methods: The Surveillance, Epidemiology, and End Results database was queried to identify patients with primary cardiac sarcoma from 1973 to 2015. Kaplan-Meier analysis and log-rank tests were performed to compare overall survival for subpopulations, stratified on year at diagnosis (dichotomized into 2 periods: 1973-2005 and 2006-2015), pathological types, whether patients were treated with surgery or not, and surgery and chemotherapy combinations. Multivariable Cox regression was performed to estimate the adjusted hazard ratios and 95% confidence intervals of potentially clinically important factors., Results: A total of 442 patients (mean age, 47.2 ± 18.7 years; male 52.0%) were identified. Most patients were white (78.1%) and diagnosed at age 20 to 60 years (70.2%). Angiosarcoma (43.2%) was the most common histologic type. Overall, the median survival was 7 months, and the 1-, 3-, and 5-year survivals were 40.7%, 15.6%, and 9.8%, respectively. Patients who were diagnosed within the recent decade (2006-2015) did not achieve a better overall survival (P = .13). Surgery (adjusted hazard ratio, 0.49; 95% confidence interval, 0.37-0.64; P < .001) and chemotherapy (adjusted hazard ratio, 0.70; 95% confidence interval, 0.54-0.92; P = .009) were independently associated with improved overall survival. Increasing age (adjusted hazard ratio of 5-year increment, 1.07; 95% confidence interval, 1.04-1.11; P < .001) was independently associated with worse survival., Conclusions: At the population level, primary cardiac sarcoma has a poor prognosis. Both surgery and chemotherapy are associated with improved survival, whereas increasing age at diagnosis was associated with worse survival., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is widely used to identify cardiac neoplasms, for which diagnosis is predicated on enhancement stemming from lesion vascularity: Impact of contrast-enhancement pattern on clinical outcomes is unknown. The objective of this study was to determine whether cardiac metastasis (C MET ) enhancement pattern on LGE-CMR impacts prognosis, with focus on heterogeneous lesion enhancement as a marker of tumor avascularity., Methods: Advanced (stage IV) systemic cancer patients with and without C MET matched (1:1) by cancer etiology underwent a standardized CMR protocol. C MET was identified via established LGE-CMR criteria based on lesion enhancement; enhancement pattern was further classified as heterogeneous (enhancing and non-enhancing components) or diffuse and assessed via quantitative (contrast-to-noise ratio (CNR); signal-to-noise ratio (SNR)) analyses. Embolic events and mortality were tested in relation to lesion location and contrast-enhancement pattern., Results: 224 patients were studied, including 112 patients with C MET and unaffected (C MET -) controls matched for systemic cancer etiology/stage. C MET enhancement pattern varied (53% heterogeneous, 47% diffuse). Quantitative analyses were consistent with lesion classification; CNR was higher and SNR lower in heterogeneously enhancing C MET (p < 0.001)-paralleled by larger size based on linear dimensions (p < 0.05). Contrast-enhancement pattern did not vary based on lesion location (p = NS). Embolic events were similar between patients with diffuse and heterogeneous lesions (p = NS) but varied by location: Patients with right-sided lesions had threefold more pulmonary emboli (20% vs. 6%, p = 0.02); those with left-sided lesions had lower rates equivalent to controls (4% vs. 5%, p = 1.00). Mortality was higher among patients with C MET (hazard ratio [HR] = 1.64 [CI 1.17-2.29], p = 0.004) compared to controls, but varied by contrast-enhancement pattern: Diffusely enhancing C MET had equivalent mortality to controls (p = 0.21) whereas prognosis was worse with heterogeneous C MET (p = 0.005) and more strongly predicted by heterogeneous enhancement (HR = 1.97 [CI 1.23-3.15], p = 0.005) than lesion size (HR = 1.11 per 10 cm [CI 0.53-2.33], p = 0.79)., Conclusions: Contrast-enhancement pattern and location of C MET on CMR impacts prognosis. Embolic events vary by C MET location, with likelihood of PE greatest with right-sided lesions. Heterogeneous enhancement-a marker of tumor avascularity on LGE-CMR-is a novel marker of increased mortality risk.

Introduction: Primary benign right ventricular tumours are rare. They can cause significant mortality without appropriate and timely treatment. We investigated surgical treatment and survival characteristics for right ventricular tumours., Materials and Methods: From 2007 to 2017, 21 patients with primary benign right ventricular tumours who underwent tumour resection were retrospectively reviewed. Clinical findings and follow-up results were analysed., Results: Thirteen men and eight women were enrolled, with a mean age of 42.3 ± 15.3 years. The most frequent histotypes were myxoma, haemangioma and papillary fibroelastoma. Eight patients underwent concomitant tricuspid valvuloplasty and one had tricuspid valve replacement. No major adverse events or death occurred during the perioperative period. One patient with haemangioma underwent partial tumour resection; however, the tumour regressed gradually during follow-up. Within the 10-year follow-up period (mean 4.8 ± 2.6 years), the recurrence-free and overall survival rates were 81.0% and 85.7%, respectively., Conclusions: Tumour resection for primary benign right ventricular tumours is safe and effective, and has a good prognosis. Tricuspid valvuloplasty or tricuspid valve replacement may be necessary for the resection of right ventricular tumours to improve the haemodynamics. Haemangiomas naturally undergo spontaneous regression.

Background: Cardiac metastases (CM) from neuroendocrine tumours (NET) are rare; however, with the introduction of new molecular imaging modalities, such as 68Ga-DOTATATE PET-CT for NET diagnosis and re-staging, they are now identified more frequently. This study presents a single-institution experience on the NET CM characteristics, management, and prognostic implications., Methods: Between January 1998 and January 2020, 25 NET patients with CM were treated in our unit. A retrospective review of electronic records was performed. Overall survival (OS) was assessed by the Kaplan-Meier method. Cox regression models were used to evaluate the association of various clinical variables with OS., Results: The median age in the NET CM cohort was 64 years, with small intestine being the most common primary (84%). Nearly half of the patients suffered either from shortness of breath (48%) or had palpitations (12%). Peptide receptor radionuclide therapy (PRRT) was applied in more than half of the patients (64%), who had an improved trend for a longer median OS compared to those patients who did not receive PRRT (76.0 vs. 14.0 months, p = 0.196). The multivariate analysis demonstrated that concomitant skeletal or pancreatic metastases, as well as N-terminal pro-B-type natriuretic peptide (NT pro-BNP) >2 × upper limit of normal (ULN), were independent poor prognosticators., Conclusions: Clinical features of NET CM ranged from asymptomatic patients to heart failure. Concomitant bone or pancreatic metastases and NT pro-BNP levels >2 ULN predicted shorter survival time. PRRT serves as a feasible therapy with promising survival benefits; however, more data are needed., (© 2021 S. Karger AG, Basel.)

The article presents a case of sudden death of a 56-year-old woman at the workplace, caused by a very rare primary cardiac tumor. The patient's family reported a crime to the prosecutor's office suggesting participation of third parties in causing the death or malpractice in physical examinations before the death. A review of clinical data concerning cardiac angiosarcoma, available in electronic databases (e.g., Web of Science, PubMed), was presented, which could be useful in the practice of occupational medicine specialists. A legal analysis of potential claims to occupational medicine specialist in the case of failure to recognize primary cardiac tumors was also included in the article. Med Pr. 2020;71(6):757-64., (This work is available in Open Access model and licensed under a CC BY-NC 3.0 PL license.)

Background: Primary cardiac sarcoma (PCS) is a deadly disease. The impacts of tumour size on prognosis and surgical outcomes in PCS patients remains unclear. Here, we evaluate the impact of tumour size on overall survival (OS) and cancer-specific survival (CSS) of PCS patients to provide a reference for the surgical treatment., Methods: A total of 261 PCS participants enrolled from 1983 to 2016 were identified from the Surveillance, Epidemiology, and End Results database. Using the X-tile program, we classified the tumour size into 2 subgroups: ≤ 4.0 cm and > 4.0 cm. The Kaplan-Meier method was used to determine OS and CSS. Univariate and multivariate Cox regression analyses were used to identify the independent prognostic impacts of tumour size and surgery in the 2 subgroups (≤ 4.0 cm vs > 4.0 cm)., Results: With the use of 4.0 cm as a cutoff value, tumour size seemed to be an independent prognostic factor for OS (P = 0.009) and CSS (P = 0.014) of PCS patients. Surgery improved the OS (P = 0.017) and CSS (P = 0.040) in PCS patients with tumour size > 4.0 cm but not in with tumour size ≤ 4.0 cm (both P > 0.05)., Conclusions: Tumour size of > 4.0 cm is an independent predictor of poor prognosis and is associated with the surgical outcomes in PCS patients. Surgery significantly improves the prognosis in PCS patients with tumour size > 4.0 cm. Our findings have the potential to assist clinicians to better evaluate the prognosis of PCS patients and develop optimal therapeutic strategies., (Copyright © 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

This study aimed to evaluate the incidence and prognosis of primary cardiac lymphoma (PCL) by using the Surveillance, Epidemiology, and End Results Program (SEER) database. Patients diagnosed with PCL and the disease incidence in the SEER database from 1975 to 2016 were included. Overall survival (OS) and cause-specific survival (CSS) curves were calculated using the Kaplan-Meier method and compared by the log-rank test. Univariate and multivariable Cox proportional hazard regression analyses were used to identify associations with outcome measures. The incidence of PCL was 0.011/100 000, and a predominance of elderly and male patients was observed. A total of 144 patients were enrolled. The median age of onset was 68 (9-96) years, including 80 (55.6%) males and 64 (44.4%) females. Multivariate analysis revealed that age and chemotherapy were independent prognostic factors for OS (both P < .05). Ann Arbor stage and chemotherapy were independent prognostic factors for CSS (both P < .05). In terms of treatment modality, chemotherapy combined with surgery was an independent protective factor for OS and CSS (both P < .05). For patients with primary cardiac diffuse large B-cell lymphoma (cardiac DLBCL), multivariate analysis also showed that age, Ann Arbor stage, and chemotherapy were all independent prognostic factors for OS and CSS (all P < .05). Chemotherapy combined with surgery was associated with a significant benefit in terms of OS and CSS (both P < .05). Our study confirmed that older age and advanced Ann Arbor stage were independent risk factors for PCL, and treatment with chemotherapy or cooperation with surgery resulted in better long-term survival., (© 2020 John Wiley & Sons Ltd.)

The heart is a rare primary site of lymphoma, and cardiac involvement is thought to bring a poorer prognosis. A framework of known clinical presentations, diagnostic features, disease complications, treatments, and outcomes to improve prognostication was constructed by a systematic review in 2011. However, some aspects must be discussed further in light of recent advances in lymphoma research. We collected cardiac lymphoma case reports published from January 2009 to January 2019, collected statistics from each patient, and performed a systematic analysis. The epidemiological characteristics, clinical manifestations, treatments, responses, and survival of primary cardiac lymphoma (PCL) patients are described. We obtained 158 cases of heart lymphoma, of which 101 were defined as PCL. There were more male than female cases. Most cases were diffuse large B-cell lymphoma. Six cases of PCL in cardiac myxomas were described. Patients with arrhythmia had shorter progression-free survival compared those without (HR 0.334, 95% CI 0.112-0.999, log-rank P = 0.042). Surgery did not improve patients' long-term prognosis or reduce the risk of death within 1 month. These data suggest that central nervous system prophylaxis is necessary. The overall survival was longer than that in data from 1949 to 2009.

Background No data are available on sex disparities in prevalence and survival for primary malignant cardiac tumors (PMCT). This study aimed to compare male and female PMCT prevalence and long-term survival rates. Methods and Results We utilized the Surveillance, Epidemiology, and End Results (SEER) 18 database from the National Cancer Institute for all PMCTs diagnosed between 1973 and 2015. From a total of 7 384 580 cases of cancer registered in SEER, we identified 327 men and 367 women with PMCTs. The majority (78%) of patients were white. Sarcoma was the most common type of PMCT in both men and women (≈60%). Individuals diagnosed with lymphoma exhibited better survival than those with other types of PMCTs. Men were diagnosed at a younger age than women; however, there was no significant difference in overall survival between the sexes. Men diagnosed with PMCT between the ages of 51 and 65 years demonstrated prolonged survival compared with those diagnosed at younger or older ages. There was no difference in survival rates among women based on age at diagnosis. Conclusions PMCTs are rare in both men and women. Tumors tend to be diagnosed at an earlier age in men compared with women, but there is no sex disparity in survival rate. Sarcoma is the most common type of PMCT, and lymphoma is associated with the highest survival rate among both sexes.

Background: Data on primary cardiac malignancies are limited to small single-center studies., Objectives: The aim of the current study was to provide detailed outcomes for treatment of primary cardiac malignancies from a multi-institutional database., Methods: Outcomes were acquired from the National Cancer Database for all solid primary cardiac malignancies from 2004 to 2016. The primary outcome was long-term survival. Logistic regression was used to determine factors associated with mortality., Results: A total of 100,317 cardiac tumors were identified, of which 826 (0.8%) were primary malignant tumors. After exclusion criteria, the cohort consisted of 747 patients (median age 53 years, 47.5% women). Most tumors were primary sarcomas (88.5%), the majority of which were hemangiosarcoma (40.4%). A total of 136 patients received no therapy, 113 received just chemotherapy, and 20 received just radiation. Surgery was performed in 442 (59.2%) patients including 255 patients undergoing multimodal therapy (surgery with chemotherapy, radiation, or chemoradiation). With surgery alone, 90-day mortality was 29.4%. Overall 30-day, 1-year, and 5-year survival rates were 81.2%, 45.3%, and 11.5%, respectively. The surgery group as compared with the no surgery groups had significantly better long-term survival (p < 0.0001). For stage III disease, there was a statistically significant improvement in survival with the addition of chemotherapy to surgery., Conclusions: Primary cardiac malignancies are rare cancers with dismal long-term survival despite mode of treatment. Patients who underwent surgery and those with stage III disease who received peri-operative chemotherapy had better survival compared with those who did not. However, there was likely a significant selection bias in patients chosen for surgical or medical therapy., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

To evaluate the contribution and performance of multimodal imaging in the diagnostic and therapeutic management of cardiac masses. We carried out a monocentric retrospective study on patients referred for cardiac mass assessment between 2006 and 2019, and analyzed the respective contribution of transesophageal echocardiography (TEE), cardiac computed tomography (CT), cardiac magnetic resonance (CMR) and 18 F-fluorodeoxyglucose positron emission tomography coupled with CT ( 18 F-FDG PET-CT). For each test, we determined strategy before and after its completion (need for another imaging or decision-making) as well as result on benign, malignant or indeterminate nature. For the 119 patients included, all imaging modalities increased decision-making rates, which rose from 2 to 54%, 23 to 62%, 31 to 85% and 49 to 100% before and after TEE, CT, CMR and 18 F-FDG PET-CT, respectively (P < 0.001 before vs. after). TEE was particularly efficient for atrial masses, especially for the left atrium, with a decision rate rising from 0 to 74% (P < 0.001). 18 F-FDG PET-CT was the most efficient to differentiate benign and malignant etiologies (area under the curve 0.89 ± 0.06 and 0.94 ± 0.05 for benign and malignant, respectively, P < 0.001). A benign or undetermined result on each modality was associated with a good prognosis, compared to malignant. All modalities studied are useful for cardiac mass decision-making. First-line TEE is particularly efficient for atrial masses, whereas CT and CMR are useful for ventricular masses or suspicion of malignancy. A benign or malignant result for each modality is correlated to survival and 18 F-FDG PET-CT is the most effective to define it.

Objectives: Cardiac tumors and their associated outcomes are poorly characterized. This study sought to comprehensively assess the epidemiology and natural history of primary and secondary malignant cardiac tumors (PMCT and SMCT), a well as establish predictors of mortality., Methods: A comprehensive literature review was performed to identify articles reporting on PMCTs and SMCTs. The prevalence of important cardiac tumor (CT) subtypes was evaluated and further stratified based on the continental region. Outcomes of interest included short- and long-term mortality and utilization of heart transplantation (HTX). A random effect model was adopted, and a meta-regression was performed to determine predictors of the prevalence of CTs as well as predictors of operative mortality., Results: Of the 1,226 retrieved articles, 74 were included in our study (n = 8,849 patients). The mean follow-up was 2.27 years, mean age was 42.9 years, and 55% of the patients were females. There was a total number of 7,484 benign primary cardiac tumors (PCTs) (5,140 were myxoma), 862 (9.7%) malignant PCTs, and 355 secondary cardiac tumors. The prevalence of PMCTs among PCTs was 10.83% [95%CI = 09.11; 12.83%] with a trend towards being lower in South America compared to other continents (Prevalence = 5.80%). The prevalence of HTX among all patients was 2.45% [1.36; 4.38%]. The pooled short-term mortality was 5.90% [4.70; 7.39%] and the incidence of late mortality in all CTs, benign CT and PMCTs was 2.55% [1.76; 3.72%], 0.79% [0.46; 1.37%] and 14.77% [9.32; 23.40%], respectively. On meta-regression, the annual volume of cardiac tumor cases per center was the only predictor of lower early mortality (Beta = -0.14 ± 0.03, P < 0.0001)., Conclusions: PMCTs represent the minority of PCT (~10%) and have a higher prevalence in Europe and North America. Survival is higher in benign pathology and is significantly improved by treatment in specialized high-volume centers. Approximately 2% of patients with CTs undergo heart transplantation., Competing Interests: Declaration of competing interest None., (Published by Elsevier Ltd.)

Background: Primary malignant cardiac tumors (PMCTs) are fatal, but up to now, there is still a lack of survival prediction model for prognosis evaluation. We developed nomograms to predict overall survival (OS) and cancer-specific survival (CSS) for PMCTs by the Surveillance, Epidemiology, and End Result (SEER) database., Methods: A total of 506 PMCTs participants were identified in the SEER database from 1973 to 2014 and were randomly assigned into the training cohort (N = 354) and the validation cohort (N = 152). The prognostic factors for PMCTs were identified by Kaplan-Meier and multivariate Cox analysis and further incorporated to build OS and CSS nomograms. The nomograms were internally and externally validated via concordance indexes (C-index) and calibration curves., Results: The independent prognostic factors for OS and CSS in PMCTs were associated with age at diagnosis, histopathology, tumor stage, cancer-directed surgery, and chemotherapy (all P < .05). In the internal validation, the C-index values were 0.71 (95% confidence interval [CI]: 0.68-0.75) for OS nomogram, and 0.70 (95% CI: 0.67-0.74) for CSS nomogram. In the external validation, the C-index values were 0.71 (95% CI: 0.66-0.77) for OS nomogram, and 0.71 (95% CI: 0.65-0.77) for CSS nomogram. The calibration curves of internal and external validation showed consistency between the nomograms and the actual observation. The risk stratification of PMCTs was significant distinction (P < .05)., Conclusion: We developed and validated credible nomograms to predict OS and CSS in PMCTs. These nomograms can be offered to clinicians to more precisely estimate the survival and identify risk stratification of PMCTs., (© 2019 Wiley Periodicals, Inc.)

Background:  Cardiac metastases are more common than primary malignant tumors of the heart and are usually treated surgically as a palliative approach. In this study, we reviewed our experience with the surgical treatment of patients with cardiac metastases of various types of malignant tumors., Methods:  Between 2003 and 2016, 10 patients underwent surgery for cardiac metastases at our institution., Results:  The mean age was 53.5 ± 19 years. Female patients made up 60% ( n  = 6) of the collective. The cohort included cardiac metastases of diverse origins (peripheral sarcomas, melanoma, rectal carcinoma, and hepatocellular carcinoma). The left side of the heart was more frequently affected ( n  = 7). In only six patients, the primary malignancy was known at the time of cardiac surgery. The interval between the first diagnosis of the primary tumor and cardiac metastases ranged from simultaneous diagnosis to up to 19 years. At the time of the diagnosis of the cardiac metastases, seven patients already had multiple metastases: all seven patients had pulmonary metastases, and three of them additionally had hepatic, cerebral, or osseous metastases. Only four patients were symptomatic (atrial fibrillation, pericardial effusion, tachycardia with chest pain, dyspnea). There was no in-hospital death. The postoperative course was uneventful overall. The one- and two-year survival rates were similar, that is, 76%. The median follow-up time was 5.4 years., Conclusions:  Surgical intervention for treating cardiac metastases is associated with uneventful clinical outcome and acceptable survival in this critically ill population. Control of the primary malignancy, and maybe other metastases, determines the survival., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background:  Primary cardiac sarcoma (CS) is an extremely rare disease. This study aims to identify possible prognostic factors for long-term survival., Methods:  A total of 17 consecutive patients who were treated for primary CS between 2003 und 2018 at two cardiac centers were investigated. Clinical data and histological characteristics of the tumors were analyzed. Long-term follow-up of all patients were performed., Results:  The median age was 54 years (range: 23-74). The tumors originated from the left side of the heart in nine patients. Histologically, there were four angiosarcomas, three intimal sarcomas, and three synovial sarcomas. One- and 7-year survivals were 81.9 and 18.2%, respectively. Low expression levels of K i -67 tended to be associated with increased survival (log-rank p  = 0.06). Adjuvant chemotherapy but not radiotherapy regardless of existing metastases was associated with significantly increased survival (log-rank p  = 0.001)., Conclusion:  Angiosarcoma was the most common type of CS. The survival of CS patients is poor but prognostic factors, such as K i -67, may help estimate the course of the disease. Survival could be improved significantly with chemotherapy., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Cardiac synovial sarcoma (CSS) is an extremely rare malignant tumor with a severe prognosis, due to frequent relapses and metastases. To obtain useful information for treatment protocols, we analyzed survival and therapy data from the cases reported in the literature., Methods: A search of MEDLINE was performed throughout December 2018. Using key words relating to primary CSS, we collected from the literature a total of 97 cases, mainly consisting of single case reports. To identify predictors of overall survival, statistical analyses were performed on a selected cohort of 55 patients for whom relevant clinicopathological data were available, including surgery and adjuvant therapy., Results: The univariable analysis revealed that patients in their first three decades of life have better overall survival. The univariable analysis also showed that patients not receiving adjuvant chemotherapy are at increased risk of death. In the multivariable analysis, tumor resection and chemotherapy are factors significantly improving overall survival., Conclusion: The survival of patients with CSS is positively influenced by a young patient's age and greatly improved by the administration of chemotherapy, even in the absence of tumor resection., (© 2019 Wiley Periodicals, Inc.)

The literature on malignant cardiac tumors is relatively limited because they are rare, especially among the Chinese population. We analyzed 14 patients diagnosed with malignant cardiac tumors in Fuwai Hospital and present the results of surgical treatments on the tumors. The mean age at tumor diagnosis was 47 years in a male-dominated cohort. There was a high frequency of pericardial effusion and coronary artery involvement in our group. We compared the survival times of patients who received different treatments and found that surgery improved prognosis of tumors, especially for patients who underwent orthotopic heart transplantation.

Background Cardiac magnetic resonance ( CMR) differentiates neoplasm from thrombus via contrast enhancement; positron emission tomography ( PET) assesses metabolism. The relationship between CMR contrast enhancement and metabolism on PET is unknown. Methods and Results The population included 121 cancer patients undergoing CMR and 18 F-fluorodeoxyglucose ( 18 F- FDG) - PET , including 66 with cardiac masses and cancer-matched controls. Cardiac mass etiology (neoplasm, thrombus) on CMR was defined by late gadolinium enhancement; PET was read blinded to CMR for diagnostic performance, then colocalized to measure FDG avidity. Of CMR -evidenced thrombi (all nonenhancing), none were detected by PET . For neoplasm, PET yielded reasonable sensitivity (70-83%) and specificity (75-88%). Lesions undetected by PET were more likely to be highly mobile ( P=0.001) despite similar size ( P=0.33). Among nonmobile neoplasms, PET sensitivity varied in relation to extent of CMR -evidenced avascularity; detection of diffusely enhancing or mixed lesions was higher versus predominantly avascular neoplasms (87% versus 63%). Colocalized analyses demonstrated 2- to 4-fold higher FDG uptake in neoplasm versus thrombus ( P<0.001); FDG uptake decreased stepwise when neoplasms were partitioned based on extent of avascularity on late gadolinium enhancement CMR ( P≤0.001). Among patients with neoplasm, signal-to-noise ratio on late gadolinium enhancement CMR moderately correlated with standardized uptake values on PET ( r=0.42-0.49, P<0.05). Mortality was higher among patients with CMR -evidenced neoplasm versus controls (hazard ratio: 1.99 [95% CI, 1.1-3.6]; P=0.03) despite nonsignificant differences when partitioned via FDG avidity (hazard ratio: 1.56 [95% CI, 0.85-2.74]; P=0.16). Among FDG-positive neoplasms detected concordantly with CMR , mortality risk versus cancer-matched controls was equivalently increased (hazard ratio: 2.12 [95% CI, 1.01-4.44]; P=0.047). Conclusions CMR contrast enhancement provides a criterion for neoplasm that parallels FDG -evidenced metabolic activity and stratifies prognosis. Extent of tissue avascularity on late gadolinium enhancement CMR affects cardiac mass identification by FDG - PET .

Objective: Cardiac paragangliomas are rare neuroendocrine tumors. Early surgical treatment improves clinical symptoms and prolongs survival. We review our experience in 17 patients who underwent surgical resection for functional cardiac paraganglioma., Methods: Seventeen patients underwent surgery for functional cardiac paraganglioma from 2004 to 2017 were identified. Clinical data and long-term outcomes were extracted and analyzed., Results: All 17 patients with cardiac paraganglioma (11 males) with a median age of 35 years (range, 11 to 51 years) were hormonally functional and underwent operations. A 24-hour urine catecholamine assay documented elevation of norepinephrine, epinephrine, and dopamine. Tumors were determined with octreotide scintiscan in all 17 patients and metaiodoben-zylguanidine scintigraphy in 10 of 15 patients. Tumor sites were right atrioventricular groove in 4 patients, between the main arteries in 10 patients, and interatrial groove in 4 patients. All patients underwent complete resection. Concomitant surgeries were: structural reconstruction in 16 patients (94%) and coronary artery bypass graft in 8 patients (47%). One patient had a history of incomplete resection of an intra-atrial tumor. One patient died postoperatively. Operative mortality is 5.8%. During follow-up (mean, 6.5 years; range, 1.5 to 14.2 years), 16 patients are alive in functional class I or II. Two patients developed tumor recurrence and 14 remained symptom-free with normal urinary catecholamines., Conclusions: With a multidisciplinary treatment, early diagnosis, complete resection for the tumor along with complex reconstruction is achievable for most patients, and it offers promising long-term survival., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Cardiac tumors are uncommon, occurring in less than 1% of the population, and are comprised of numerous tumor types. Management of certain tumors types such as sarcoma have evolved and improved in the recent era. We evaluate the outcomes of patients who underwent resection of benign or malignant cardiac tumors with a focused review of cardiac sarcomas., Methods: Institutional data were reviewed from 1997 to 2017, and 180 patients who underwent tumor resection were identified. Outcomes and survival were examined based on tumor type., Results: Two-thirds of patients (119 of 180) had benign tumors. Of 61 malignant tumors, 23 were sarcomas, 24 were cavoatrial tumors, and 8 were T4 lung tumors. In the sarcoma group, operative mortality was 2 of 23 (9.1%). Neoadjuvant therapy was administered to 8 of 23 patients (34.8%) with R0 resection achieved in 5 of 8 patients (62.5%). R0 resection was successful in 7 of 15 patients (46.7%) without neoadjuvant therapy. Mean survival with neoadjuvant therapy was 2.76 ± 3.85 years versus 1.28 ± 1.31 years without neoadjuvant therapy (p = 0.428). Mean survival with R0 resection was 2.79 ± 4.23 years compared with 1.64 ± 1.63 years without (p = 0.407). In the T4 lung tumor group, operative mortality was zero and R0 resection was achieved in 6 of 8 (75%). The cavoatrial tumors were mostly renal cell carcinoma resected with a mortality of 4.5%., Conclusions: Cardiac tumors are comprised of diverse tumor types. Indications for, and benefits of, resecting benign tumors and many malignant tumor types are clear, and operative outcomes are generally good. Cardiac sarcomas benefit from neoadjuvant therapy, which improves the rate of complete resection, thus improving survival., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Primary pericardial mesothelioma (PPM) is a rare cancer for which there is no consensus on treatment. We evaluated and summarized a large contemporary population of published PPM cases to characterize risk factors, treatment patterns, and clinical outcomes. Using Ovid and PubMed, literature published from 2000 through 2016 was searched using the terms "primary pericardial mesothelioma," "pericardial mesothelioma," and "malignant pericardial mesothelioma." We identified 6 case series and 84 case reports for a total of 103 PPM cases published from 2000 through 2016. The median age at diagnosis was 55 years, and the median overall survival was 6 months. In univariate analyses of clinical characteristics including gender, asbestos exposure, tobacco use, prior radiation exposure, histologic subtype, and metastasis and/or mediastinal spread, only the presence of metastasis and/or mediastinal spread was a significant predictor of decreased survival (P = .015). Surgery did not provide a statistically significant survival benefit (P = .12). A survival benefit was noted in those who received chemotherapy (median survival, 13 months vs. 0.5 months, P = .002), specifically chemotherapy with a platinum agent with or without pemetrexed. In multivariate analysis, only the receipt of chemotherapy was associated with improved survival. PPM remains a rare and poorly understood malignancy with unclear etiology and a poor prognosis. In this retrospective systematic review, a survival benefit was seen in patients who received chemotherapy., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Primary cardiac tumors are rare, but few studies have examined the relationship between risk factors and the prognosis. The aim of this study was to provide a survival analysis and risk factors for mortality in patients with primary cardiac tumors., Methods: We retrospectively enrolled 71 patients diagnosed with primary cardiac tumors from June 2006 to November 2017 in our hospital. Patients' population characteristics, treatment information, pathology, and follow-up data were obtained and analyzed., Results: Of the 71 patients, 60 cases were benign, and 11 cases were malignant. Sex, age, New York Heart Association classification, the percentage of peripheral embolism, and surgery had no significant difference between benign and malignant groups (P >.05), but the percentage of arrhythmia, leg edema, and mortality rate was higher in the malignant tumor group than in the benign tumor group (P <.05). Compared with the benign tumor group, the percentage of biatrial lesions in the malignant tumor group was significantly higher (P <.05). Moreover, Independent risk factors included the treatment choice, pathology type, and number of tumor lesions (P <.05)., Conclusion: Our study suggests that conservative therapy, malignant cardiac tumor, and biatrial tumor lesion are independent risk factors for poor prognosis., (2019 Forum Multimedia Publishing, LLC)

Objective: Primary cardiac sarcomas (PCS) are extremely rare, portend a very poor prognosis, and have limited outcomes data to direct management. This study evaluated the impact of postoperative chemotherapy and/or radiotherapy on survival for PCS., Methods: A retrospective chart review was conducted of 12 patients diagnosed with and who underwent resection for PCS at a single institution between 2000 and 2016. Data were collected on patient/tumor characteristics and analyzed with respect to treatment and outcome using Kaplan-Meier methods., Results: Median age was 43 (range 21-73 years) with a 50:50 male-to-female ratio. The most common subtype was angiosarcoma (42%), and 25% presented with distant metastases (DMs). The initial treatment modality for all patients was surgery, with 58% having macroscopically positive (R2) margins. In total, 75% received postoperative chemotherapy and/or radiotherapy. Median progression-free survival (PFS) was 5.9 months, and median overall survival (OS) was 12.0 months. Achieving negative or microscopically positive margins (R0/R1) as compared with R2 resection significantly improved PFS (12.6 vs 2.7 months, P = .008) and OS (21.8 vs 7.2 months, P = .006). DM at presentation demonstrated a significantly shorter OS (7.0 vs 16.9 months, P = .04) and PFS (0.7 vs 7.9 months, P = .003) compared with localized disease. Patients given postoperative therapy had longer OS compared with surgery only, but this difference was not statistically significant (15.5 vs 2.6 months, P = .12)., Conclusions: Gross total surgical resection can significantly improve PFS and OS in PCS, but DM at diagnosis is an extremely poor prognostic sign. Postoperative therapy should be considered, although this study was likely underpowered to demonstrate a statistically significant benefit., (Copyright © 2018. Published by Elsevier Inc.)

Objectives: To investigate risk factors contributing to early death in patients diagnosed with primary malignant cardiac tumors (PMCTs) and derive better understanding of these poorly characterized individuals., Method: Data from the Surveillance, Epidemiology and End-Results (SEER) registries on 564 patients diagnosed with PMCTs between 1973 and 2014 were analyzed. Early death was defined as survival of ≤3 months from the time of diagnosis. Two-tailed χ 2 or fisher's exact test were used for association between categorical variables and occurrence of early death. Logistic regression analysis was used to assess independent risk factors of early death. Time trends in early death rates of PMCTs were described using scatter plot., Results: Of the 564 patients with PMCTs, early death was identified in 214 individuals (37.9%). Patients with unspecified soft tissue sarcomas and blood vessel tumors had the highest risk of early death. Age > 80 years and non-consent for surgery were strong predictors of early death in all PMCT subtypes. In sarcomas, disadvantaged income was associated with an increase in early mortality, while black race was associated with a reduction in early mortality. In mesotheliomas and others, male sex was a risk factor for early mortality, while Hispanic ethnicity was associated with a reduction in early mortality. Percentages of early death slightly decreased over the past 40 years., Conclusions: Predictors of early death are primarily related to age older than 80 years, no surgery and specific histopathology types but also include disadvantaged socioeconomic status and male sex. Initiatives to identify those at risk and develop preventive interventions should be prioritized., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Primary malignant cardiac tumors rarely occur in clinical care. Little is known about the impact of a parameter on postoperative survival., Methods: From May 1991 to May 2014, a total of 24 patients underwent surgical treatment of a primary cardiac sarcoma in our center. We analyzed our clinical database retrospectively for information on patient characteristics and treatment data. The follow-up could be completed to 91.7%., Results: Angiosarcoma and non-otherwise-specified sarcoma were the most common tumor entities. R0 resection was achieved in most cases. Postoperative mortality within the first 30 days was 20.8% ( n  = 5). In four of these five cases, postoperative low-output cardiac failure was the leading cause of death. The cumulative survival rate was 77.3% after 30 days, 68.2% after 3 months, 50.0% after 6 months, 45.0% after 12 months, and 18.0% after 24 months. The mean survival time in the whole group was 47.0 months. A low tumor differentiation was associated with low mean survival, but this was not statistically significant. Mean survival of sarcoma was higher after R0 resection. There was no significant rate of survival difference regarding the adjuvant therapy concept., Conclusion: Extended surgery alone or in combination with chemo- and/or radiotherapy may be successful in certain cases and may offer a satisfactory quality of life. The establishment of a multicenter heart tumor register in Germany is necessary to increase the number of cases in studies, get more remarkable study results, and standardize the diagnosis and therapy., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Although ventricular fibromas are rare, they are the second most common type of cardiac tumor in children. While histologically benign, they have a propensity to cause malignant arrhythmias, with cardiac arrest often being the first presentation., Objective: The purpose of this study was to evaluate the arrhythmia risk and management strategies for pediatric ventricular fibromas., Methods: Fifteen centers in the British Paediatric Arrhythmia Group network were contacted to partake in the study to contribute cases. A detailed database search was performed at 2 hospitals for cases of ventricular fibromas., Results: A total of 19 patients were included in the study. Arrhythmias were common, with 5 patients presenting with cardiac arrest and 5 others having documented ventricular tachycardia. Nine patients have undergone surgical resection at various hospitals, and all these patients have survived with good long-term outcomes. One patient who did not have any treatment died, presumably of a ventricular arrhythmia; another died of metastatic disease. There were no recurrences of arrhythmia after surgery, and the need for a defibrillator was alleviated in all cases., Conclusion: Ventricular fibromas have a high propensity to cause malignant arrhythmias, and if they are not managed appropriately, mortality is high. The outcomes of surgical resection are good, regardless of size, and this represents the best therapeutic option, with most patients being symptom free in the longer term., (Copyright © 2018 Heart Rhythm Society. All rights reserved.)

Cardiac myxoma (CM) is the most common benign cardiac tumor. We retrospectively reviewed our single center experience in 153 patients with CM over a period 25 years.From November 1993 to May 2017, 153 patients were operated in our institution with diagnosis of a CM. In all patients preoperative, perioperative, and postoperative data were recorded including the long-term follow-up. All patients followed up in the outpatient's clinics and echocardiography at regular intervals.Mean age 59 ± 12 years old. There were 104 women and 49 men. Preoperative clinical manifestations of the patients were hemodynamic consequences (47.7%), asymptomatic (46.4%), systemic embolism (4.5%), systemic manifestations-fever (0.7%), and hemoptysis (0.7%). The most common location of CM was in the left atrium in 82.4% patients. Mean tumors diameter was 4.5 ± 1.9 cm. In addition, we were observed that the age of the patients have differences between sex groups women versus men, 60.3 and 54.8 years old respectively (P = .02). On the other hand the tumor size have not differences between the sex groups (P = .56). Combine operations were performed in 24 (15.7%) patients. New cerebrovascular accident was observed in 2 patients post-op. Mean in-hospital stay was 8.02 ± 2.8 days. In-hospital mortality was 1 patient (0.7%) (from sepsis). During median follow-up 3.7 ± 4.3 years CM recurrence was identified in 5 (3.3%) patients.Surgical resection of CMs contributes in an excellent prognosis and associated with low complications and recurrences rate. Regular long-term follow-up is recommended in all patients with CM.

Objectives: Malignant intracardiac tumours are rare, and consensus concerning the optimal therapeutic approach is lacking. We performed a retrospective medical analysis, identifying 9 patients having been operated for cardiac sarcomas. All of them had a complete postoperative long-term follow-up. To enhance understanding of the best therapeutic approach for future patients, it is crucial to reveal special medical problems and to analyse the potential impact they may have on disease course and survival rate in this specific patient group., Methods: Cardiac tumours operated on 2000 to the end of 2015 were reviewed. Late mortality during the follow-up period was determined. The impact of tumour extension, tumour localization, resection status (complete versus partial) and histopathological diagnosis on survival was analysed retrospectively., Results: Of all cardiac malignant tumours resected, sarcomas were, with an incidence of 0.14% (9 patients), the most frequent histological group admitted to cardiac surgery. All of the patients presented with cardiac symptoms. All of the patients survived the operation and all had relief or improvement of cardiac symptoms. The mean follow-up period was 17 ± 13 months. Five patients died after 6, 8, 12, 12 and 15 months, respectively. Four survivors (3 with a pulmonary artery tumour sarcoma and 1 with a left atrial sarcoma) had a mean follow-up of 26 ± 17 months. Macroscopically complete tumour resection, absence of metastatic spread and histological sarcoma type had an impact on follow-up survival., Conclusions: Although cardiac sarcomas are rare, surgeons occasionally encounter them. A 1-year mortality rate of 44% reflects an unfavourable prognosis, but surgery seems to be a secure, reliable option in selected patients for treating cardiac symptoms and avoiding early cardiac-related deaths.

Background: In Brazil, underreporting of mesothelioma and cancer of the pleura (MCP) is suspected to be high. Records from death certificates (SIM) and hospital registers (SIH-SUS) can be combined to recover missing data but only anonymous databases are available. This study shows how common data can be used for linkage and as an assessment of accuracy., Methods: Mesothelioma (all sites, ICD-10 codes C45.0-C45.9) and cancer of the pleura (C38.4) were retrieved from both information systems and combined using a linkage algorithm. Accuracy was examined with non-anonymous databases, limited to the state of São Paulo., Results: We found 775 cases in death certificates and 283 in hospital registers. The linkage matched 57 cases, all accurately paired. Three cases, 0.4% in SIM and 1.3% in SIH-SUS, could not be matched because of data inconsistencies., Conclusions: A computer linkage can recover MCP cases from hospital records not found in death certificates in Brazil., (© 2018 Wiley Periodicals, Inc.)

Background: Primary cardiac myxofibrosarcoma is a very rare cardiac malignancy. The majority of publications are limited to case reports. No pooled analyses of primary cardiac myxofibrosarcoma cases are available. Little clinical features and outcome patterns are acknowledged. The purpose of this study is to identify the clinical characteristics and prognostic factors of primary cardiac myxofibrosarcoma., Case Presentation: A case report of primary cardiac myxofibrosarcoma was presented, and a review of English language literatures of primary cardiac myxofibrosarcomas were performed electronically. Demographics, clinicopathologic data, therapy and follow-up were summarized. The median survival time and the mean survival time were calculated by Kaplan-Meier method. Survival distribution and overall survival were figured by log-rank test and cox proportional hazards models. We present a case, and retrospectively analyzed additional 30 patients derived from 24 isolated articles. The cohort consisted of 18 male and 13 female patients. The age was 41.87 ± 17.89 years. Some common features were found in clinical presentations, pathologic features, treatments and outcome patterns of primary cardiac myxofibrosarcoma. There were special features in echocardiography, histological and immunohistochemical examinations, which should be considered in diagnosis of primary cardiac myxofibrosarcoma. The median survival time/mean survival time (MST) was 14/32.66 months. The median survival time/mean survival time (MST) was 14/32.66 months. Compared to the other groups, the following groups had shorter survival characteristics, including age ≥ 40 years (14/11.79 months), female (14/26.26 months), mass diameter ≥ 40 mm (14/14.64 months), high-grade (2/11.81 months), and no post-treatment (14/28.09 months). Statistical analyses revealed that primary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Tumors ≥ 40 mm in size (P = 0.055, HR = 6.79) or with high-grade (P = 0.063, HR = 11.45) had significantly worse prognosis., Conclusions: Primary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Echocardiography, together with histological method should be considered in ordinary diagnosis. Tumors ≥ 40 mm in size or with high-grade had significantly worse prognosis, which should be early diagnosed and treated with rational surgery.

In diagnosing cardiac and paracardiac masses, cardiac MRI (CMR) has gained acceptance as the gold standard. CMR has been observed to be superior to echocardiography in characterizing soft-tissue structures and, specifically, in classifying cardiac masses. The aim of our study was to evaluate the association between mortality and cardiac or paracardiac masses initially identified by echocardiography (ECHO) and confirmed by CMR. Between January 2002 and August 2007, a total of 158 patients underwent both ECHO and CMR for the evaluation of cardiac masses that were equivocal or undefined by ECHO. The primary study endpoints were 5-year all-cause mortality and 5-year cardiac mortality. Causes of death as of April 1, 2015 were obtained from medical records or the National Death Index. Patients were analyzed according to mass type determined by CMR using the Kruskal-Wallis test, Kaplan-Meier curves, and the log-rank test. Over a mean duration of follow-up of 10.4 ± 2.9 years (range: 0.01-12 years) post-CMR, the overall all-cause mortality rate was 25.9% (41/158). Median age at death was 76 years and there were 21 females (51.2%). Mortality rates in the different classifications of cardiac masses by CMR were as follows: 20% (1/5) in patients with a Nondiagnostic CMR; 20% (1/5) in Other Diagnoses; 17.9% (7/39) in No Masses (includes Normal Anatomical Variants); 16.7% (3/18) in Benign Masses; 23.8% (15/63) in Fat; 50% (5/10) in Thrombus; and 61.5% (8/13) in Malignant Mass. The mean survival time in patients with No Mass (n = 39) was not significantly longer than patients with any type of cardiac mass (n = 114) (P = .16). No significant difference was found in age at death between patients when grouped by CMR classification (P = .40). However, among CMR-confirmed masses, there were some significant differences by mass classification type (P = .006). During the follow-up period, 26% (41/158) of patients died and 22% (9/41) of the deaths were cardiovascular related; there was no significant difference in mean survival times with respect to cause of mortality (P = .23). In patients with cardiac masses, dually confirmed by ECHO and CMR, significant differences in survival time were observed based upon CMR classified type of mass while CMR was instrumental in obviating invasive biopsy., (© 2018 Wiley Periodicals, Inc.)

Background: Little is known about the echocardiographic characteristics of primary malignant pericardial mesothelioma (PPM) due to its rarity. The aim of this study was to explore the sex-specific echocardiographic patterns of PPM and risk factors for in-hospital mortality., Methods: A retrospective information retrieval was conducted for cases of PPM reported from China during 1981 and 2015. The diagnosis was made by histopathological examinations and only cases with echocardiographic descriptions were included. Data on the clinical and echocardiographic findings were collected. Difference in clinical, sex-specific echocardiographic characteristics and findings across different time periods were assessed. Logistic regression analysis was performed to explore echocardiographic risk factors for in-hospital mortality., Results: A total of 64 patients with PPM were included, with a mean age of 39.2 ± 15.6 years and minor male dominance (40, 62.5%). The most common echocardiographic presentations were pericardial effusion (55, 85.9%), pericardial masses (36.4%) and thickening (17.3%), respectively. The positive rate of pericardiocentesis was only 20.9%. Six patients (15.4%) died among 39 cases reporting in-hospital outcome. Logistics analysis identified no clinical or echocardiographic parameters associated with in-hospital mortality (all P > 0.05)., Conclusions: The echocardiographic signs of PPM are basically nonspecific with massive pericardial effusion as the most common sign, although no echocardiographic gender differences or association with in-hospital mortality could be identified.

Primary cardiac tumors are uncommon, and the majority of them are benign which are curable but can cause significant morbidity if not diagnosed and treated in a timely fashion. The objective of this study was to review the clinicopathologic features and surgical outcomes of patients with primary cardiac tumors in a single medical center in China. We have retrospectively reviewed 212 consecutive adult patients who underwent surgical resection of primary cardiac tumors at our center from January of 2001 to June of 2017. All available clinicopathological features, imaging characteristics, and disease outcomes were summarized and presented. The present series enrolled 180 cardiac myxomas (84.9%) and 32 non-myxoma cases that included hemangioma, lipoma, papillary fibroelastoma, schwannoma, pericardial cyst, teratoma, paraganglioma, lymphoma, undifferentiated pleomorphic sarcoma, myxofibrosarcoma, angiosarcoma, and liposarcoma. All patients were diagnosed ante-mortem. The most frequent complaint was dyspnea. Benign tumors accounted for 93.9% of cases (199/212) and malignant tumors accounted for 5.7% (12/212), and the remaining one case was intermediate (paraganglioma). The outcome of benign tumors was favorable and only three recurrent cases were documented (1.5%) after surgical resection. All the seven patients with primary cardiac sarcomas (undifferentiated pleomorphic sarcoma, myxofibrosarcoma, angiosarcoma, and liposarcoma) suffered postoperative recurrence or disease related death. Cardiac myxoma represents the most frequent primary cardiac tumors. The clinical presentations, treatment strategies and outcomes of the primary cardiac tumors depend on the tumor location and histopathological type., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: The available literature on the incidence, management and prognosis of primary malignant cardiac tumors [PMCTs] is limited to single-center studies, prone to small sample size and referral bias. We used data from the Surveillance, Epidemiology, and End Results [SEER]-18 registry (between 2000 and 2014) to investigate the distribution, incidence trends and the survival rates of PMCTs., Methods: We used SEER*Stat (version 8.3.4) and the National Cancer Institute's Joinpoint Regression software (version 4.5.0.1) to calculate the incidence rates and annual percentage changes [APC] of PMCTs, respectively. We later used SPSS software (version 23) to perform Kaplan-Meier survival tests and covariate-adjusted Cox models., Results: We identified 497 patients with PMCTs, including angiosarcomas (27.3%) and Non-Hodgkin's lymphomas [NHL] (26.9%). Unlike the incidence rate of NHL (0.108 per 10 6 person-years) that increased significantly (APC=3.56%, 95% CI, [1.445 to 5.725], P=.003) over the study period, we detected no significant change (APC=1.73%, 95% CI [-3.354 to 7.081], P=.483) in the incidence of cardiac angiosarcomas (0.107 per 10 6 person-years). Moreover, our analysis showed that the overall survival of NHL is significantly better than angiosarcomas (P<.001). In addition, surgical treatment was associated with a significant improvement (P=.027) in the overall survival of PMCTs., Conclusion: Our analysis showed a significant increase in the incidence of cardiac-NHL over the past 14 years with a significantly better survival than angiosarcomas. To further characterize these rare tumors, future studies should report data on the medical history and diagnostic and treatment modalities in these patients., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: Primary cardiac tumors are rare lesions with different histological type. We reviewed our 17 years of experience in the surgical treatment and clinical results of primary non-myxoma cardiac tumors., Methods: Between July 2000 and February 2017, 21 patients with primary cardiac tumor were surgically treated in our institution. The tumors were categorized as benign non-myxomas and malignants. Data including the demographic characteristics, details of the tumor histology and grading, cardiac medical and surgical history, surgical procedure of the patients were obtained from the hospital database., Results: Eleven patients were diagnosed with benign non-myxoma tumor (male/female:7/4), ranging in age from 10 days to 74 years (mean age 30.9±26.5 years). Papillary fibroelastoma was the most frequent type (63.6%). There were two early deaths in benign group (all were rhabdomyoma), and mortality rate was 18%. The mean follow-up period was 69.3±58.7 months (range, 3 to 178 months). All survivals in benign group were free of tumor-related symptoms and tumor relapses. Ten patients were diagnosed with malignant tumor (sarcoma/lymphoma:8/2, male/female:3/7), ranging in age from 14 years to 73 years (mean age 44.7±18.9 years). Total resection could be done in only three (30%) patients. The mean follow-up period was 18.7±24.8 months (range, 0-78 months). Six patients died in the first 10 months., Conclusion: Complete resection of the cardiac tumors, whenever possible, is the main goal of surgery. Surgical resection of benign cardiac tumors is safe, usually curative and provides excellent long-term prognosis. On the contrary, malignant cardiac tumors still remain highly lethal.

Background: Minimally invasive surgery has become the standard approach for several cardiac diseases. In this retrospective study, we compared right anterolateral minithoracotomy (RALT) with standard median sternotomy (SMS) for resection of left atrial myxoma (LAM)., Materials and Methods: From January 2009 to June 2015, the clinical data of patients who underwent RALT ( n  = 30) and SMS ( n  = 36) for resection of LAM in our hospital were collected. The preoperative clinical data and operative results were compared between the two groups., Results: There were no significant differences in aortic cross-clamp and cardiopulmonary bypass time between the two groups. The total incision length was significantly shorter in RALT group compared with SMS group ( p  < 0.001). For RALT and SMS groups, respectively, the intensive care unit length of stay was 29.2 ± 6.5 versus 43.5 ± 6.9 hours ( p  < 0.001), and the postoperative hospital length of stay was 5 days (interquartile range [IQR]: 4-6) versus 8 days (IQR: 7-10) ( p  < 0.001). The total cost in RALT group was 27,000 RMB (IQR: 25,000-29,000) versus 33,000 RMB (IQR: 31,000-35,000) in SMS group ( p  < 0.001). There were no significant differences in mortality and postoperative complications between the two groups., Conclusion: RALT approach for LAM resection can be performed safely with favorable cosmetic outcome, accepted clinical results, and lower cost. It should be considered as a promising alternative to SMS and merit additional study., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Most surgeons will encounter only a handful of primary cardiac tumors outside of myxomas. Approximately 3 quarters of primary cardiac tumors are benign and 1 quarter is malignant. In most cases, cardiac tumors are silent but when symptoms do occur, they are primarily determined by tumor size and anatomical location, not by histopathology. The diagnosis and preoperative imaging relies heavily on multimodal imaging including echocardiography, computed tomography, magnetic resonance imaging, and coronary angiography. Surgical resection is the most common treatment for most simple primary cardiac tumors and for some complex benign tumors. Surgical resection of primary cardiac tumors frequently involves the need for complex cardiac reconstruction, particularly when malignant. Secondary tumors to the heart are 30 times more frequent than primary cardiac tumors, and their incidence is increasing, largely as a result of advances in cancer diagnosis and therapy. Surgical resection is feasible in only a small fraction of highly-selected patients with secondary tumors to the heart. For complex benign tumors-such as paraganglioma or large fibromas-and all primary and secondary malignant tumors, a multidisciplinary cardiac tumor team review in experienced centers of excellence is recommended., (Copyright © 2018. Published by Elsevier Inc.)

BACKGROUND The aim of this study was to investigate the clinical presentation, operative data, and early and late outcomes of a large patient cohort undergoing surgical treatment for cardiac tumors in our institution. MATERIAL AND METHODS A total of 181 patients underwent surgery because of suspected cardiac tumor in our institution between 1998 and 2016. In 162 cases, the diagnosis was confirmed postoperatively and these patients were included in this study. Preoperative baseline characteristics, operative data, and postoperative early and long-term outcomes were analyzed. RESULTS Mean age at presentation was 56.6±17.6 years, and 95 (58.6%) patients were female. There were 126 (77.8%) patients with benign cardiac tumors, while the remaining patients had malignant tumors (primary and metastasized). The mean follow-up time was 5.2±4.7 years. The most frequent histologically verified tumor type was myxoma (63%, n=102). In terms of malignant tumors, various types of sarcomas presented most primary malignant cardiac tumors (7.4%, n=12). The mean ICU length of stay was 1.7±2.2 days and overall in-hospital mortality was 3.1% (n=5). Frequent postoperative complications included mediastinal bleeding (5.8%, n=9), wound infection (1.3%, n=2), acute renal failure (5.6%, n=9), and major cerebrovascular events (n=7, 4.6%). The overall cumulative survival after cardiac tumor resection was 94% at 30 days, 85% at 1 year, 72% at 5 years, and 59% at 15 years. CONCLUSIONS Surgical treatment of cardiac tumors is a safe and highly effective strategy associated with good early and long-term outcomes.

Gestational choriocarcinomas are highly malignant tumors with elevated serum human chorionic gonadotropin (hCG) levels. We report an extremely rare case of a 27-year-old woman who presented 4 months after normal delivery, with pulmonary, renal and intracardiac metastases of a choriocarcinoma. No primary uterine tumor was found. She was surgically treated for the renal and cardiac metastases as well as with cisplatin-etoposide chemotherapy. No recurrence has been observed 16 years after initial diagnosis, and the patient was able to have a second child. This case report shows that appropriate treatment of metastatic gestational choriocarcinoma can cure the patient without compromising her fertility.

BACKGROUND We reviewed our clinical experience with cardiac papillary fibroelastoma from 2005 to 2017. The objective of this study was to investigate the clinical and operative data, as well as the early survival rate and immediate postoperative complications. MATERIAL AND METHODS We performed a retrospective analysis of 11 patients (eight males and three females) who underwent resection of cardiac papillary fibroelastoma in our institution. RESULTS Mean age at tumor diagnosis was 60±14 years. The mean dimension of the tumor was 14±11 mm. The most common symptoms were dyspnea, palpitation, and angina pectoris, while one patient had recurrent fever attacks and another patient had a transient ischemic attack. Two patients had concomitant malignant tumors (cervical and colon carcinoma) and another two had concomitant benign neoplasms (liver cyst and thyroid adenoma). Bypass and cross clamp times were 77±32 minutes and 54±18 minutes, respectively. The tumors were found predominantly on cardiac valves (n=7). In eight cases, only tumor extirpation was performed, whereas in the other three cases, the valves had to be replaced. The mean intensive care unit length of stay was 1.1±0.3 days and there was no in-hospital mortality. All patients were alive at one-year follow-up and the survival rate was 91% in the mean follow-up period of 4.15 years. CONCLUSIONS The surgical treatment of cardiac papillary fibroelastoma was curative and safe. Thus, potential complications such as embolization or mechanical irritation of the valves can be avoided without high surgical risk.

Background: Right-side heart sarcomas tend to be bulky, infiltrative, and difficult to treat. We have previously examined our outcomes with right heart sarcomas. Surgical resection with R0 margins showed better survival than positive margins but in only one third of cases could R0 status be achieved. The hypothesis for this study was that preoperative neoadjuvant chemotherapy would shrink the tumor margins and allow an increase in R0 resection, and hence, better survival., Methods: Review of our cardiac tumor database from 1990 to 2015 yielded 133 primary cardiac sarcoma cases. Of these, we identified 44 patients with primary right-side heart sarcomas. Prospective database and retrospective data collection and clinical outcomes were evaluated for all 44 patients. Primary outcomes included 30-day mortality and morbidity and long-term survival. We used univariate and multivariate analyses to identify independent predictors of overall survival., Results: There were 27 male and 17 female patients with a mean age of 41 ± 12.7 years (range, 15 to 67). Seventy-three percent of the patients (32 of 44) received neoadjuvant chemotherapy. The most common tumor histology was angiosarcoma in 30 of 44 (68%). Thirty-day mortality was 4.5%, and statistically similar between the two groups. The median survival of patients who had R0 resection was 53.5 months compared with 9.5 months for R1. Neoadjuvant chemotherapy led to a doubling of survival (20 versus 9.5 months)., Conclusions: Neoadjuvant chemotherapy followed by radical surgery is a safe and effective strategy in patients with primary right-side heart sarcoma. This multimodality treatment enhances resectability (R0 resection) that translates into improved patient survival., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Primary cardiac tumors are rare and myxoma constitutes the majority. The present study summarizes our 30-year clinical outcomes of surgical myxoma resection., Methods: Between January 1986 and December 2015, 93 patients (30 men, 63 women; mean age, 54.7 ± 16.6 years) underwent surgical myxoma resection. The most common origin site was the left atrium. Surgery was performed via a biatrial approach in 74.2%, atrial septotomy through right atriotomy in 17.2%, and left atriotomy only in 8.6%. Mean myxoma size based on longest length was 4.73 ± 1.92 cm (range, 1.2-11.0 cm)., Results: The mean follow-up duration was 9.9 ± 7.8 years (range, 0-29 years). In-hospital mortality was 3.2%. The most common postoperative complication was atrial fibrillation (4.3%). The 5-, 10-, and 30-year survival rates were 92.9%, 87.2%, and 75.5%, respectively. Recurrence occurred in two patients (2.1%), which were detected at 20 and 79 months after the first surgery, respectively., Conclusions: Long-term survival after myxoma resection was excellent and recurrence was rare. Based on our experience, surgical method did not affect the outcome.

Background: Previous studies on primary cardiac tumors were mainly based on small case series collected from a limited number of institutions. Contemporary data of patients with primary cardiac tumors treated with or without surgery in a nationwide clinical setting are limited., Methods: Using the Diagnosis Procedure Combination database, we retrospectively identified 1317 patients hospitalized with a primary cardiac tumor (1023 myxomas, 63 non-myxomas, 72 sarcomas, 41 malignant lymphoma, 118 unspecified tumors) at 486 hospitals in Japan from July 2010 to March 2013. The outcome was overall in-hospital mortality, defined as in-hospital death occurring during the initial hospitalization or during rehospitalization. We examined the associations of baseline factors with overall in-hospital mortality and undergoing surgical resection using multivariable logistic regression analyses., Results: Overall, 914 (69.4%) patients underwent surgery and 403 (30.6%) did not. The surgery group was younger (median age, 67 years vs. 71 years, p<0.001) and was more likely to be treated at an academic hospital (38.9% vs. 27.8%, p<0.001) than the no-surgery group. The surgery group also had a higher Barthel index and a higher conscious level and showed a lower frequency of extracardiac malignancies than the no-surgery group. The likelihood of undergoing surgery was associated with coexisting cerebral infarction [adjusted odds ratio (95% confidence interval), 1.96 (1.23-3.12)] and academic hospital [1.58 (1.20-2.09)]. Patients with lower Barthel index and coexisting extracardiac malignancies were less likely to undergo surgery. Overall in-hospital mortality was 2.1% and 13.4% in the surgery and non-surgery groups, respectively. Older age, lower Barthel index, lower consciousness level, coexisting metastatic extracardiac malignancy [2.95 (1.24-7.01)], and sarcoma [21.04 (8.28-53.42)] were associated with higher overall in-hospital mortality, while academic hospital [0.41 (0.20-0.84)] and surgical resection [0.39 (0.20-0.74)] were associated with lower mortality., Conclusions: Several background factors were associated with prognosis and surgery in patients hospitalized with primary cardiac tumors., (Copyright © 2016 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)