Clinical characteristics and surgical outcomes of cardiac myxoma: A meta-analysis of worldwide experience.

Cardiac myxoma is the most common primary cardiac tumor. However, existing literature mainly consists of single-center experiences with limited subjects. This systematic review aimed to provide data on clinical characteristics and surgical outcomes of cardiac myxoma. We performed a thorough literature search on May 23, 2023 on PubMed, ProQuest, ScienceDirect, Scopus, and Web of Science. The inclusion criteria were English full-text, observational studies, and included >20 subjects. From the search, 112 studies with a total of 8150 patients were included in the analysis. The mean age was 51 years (95 % confidence interval [95%CI] = 49.1-52.3), and the majority were females (64.3 % [95 % CI = 62.8-65.8 %]). The most common clinical manifestation was cardiovascular symptoms. Echocardiography can diagnose almost all cases (98.1 % [95 % CI = 95.8-99.6 %]). Cardiac myxoma was mostly prevalent in left atrium (85.3 % [95%CI = 83.3-87 %]) and predominantly with pedunculated morphology (75.6 % [95%CI = 64.1-84.3 %]). Post-tumor excision outcomes were excellent, with an early mortality of 1.27 % (95 % CI = 0.8-1.8 %), late mortality rate of 4.7 (95 % CI = 2.5-7.4) per 1000 person-years, and recurrence rate at 0.5 (95 % CI = 0.0-1.1) per 1000 person-years. Tumor excision is warranted in a timely manner once the cardiac myxoma diagnosis is established.

Surgical experience in cardiac myxomas at a tertiary hospital.

INTRODUCTION: Cardiac myxomas account for 50% of all benign cardiac tumors. Their clinical presentation varies from embolisms to fever. Our objective was to describe the surgical experience in the resection of cardiac myxomas during an 8-year period. METHODS: This is a retrospective, descriptive study of a series of cases with cardiac myxomas diagnosed from 2014 to 2022 at a tertiary care center. Descriptive statistics were used to define the populational and surgical characteristics. We used Pearson's correlation to study the relationship between postoperative complications and age, tumor size and affected cardiac chamber. RESULTS: 31 patients were included, with a predominance of females (1:2 ratio). The prevalence was 0.44%, which was calculated based on the number of cardiac surgeries performed in our unit over the 8-year period. The main clinical manifestation was dyspnea (85%, n = 23), followed by cerebrovascular event (CVE) (18%, n = 5). Atriotomy and resection of the pedicle were performed with preservation of the interatrial septum. Mortality was 3.2%. The postoperative evolution was uneventful in 77%. Tumor recurrence occurred in 2 patients (7%), both debuting with embolic phenomena. No association was observed between postoperative complications or recurrence and tumor size, nor aortic clamping and extracorporeal circulation times with regard to age. CONCLUSIONS: Four atrial myxoma resections are performed in our unit per year, with an estimated prevalence of 0.44%. The tumor characteristics described coincide with the previous literature. A relationship between embolisms and recurrences cannot be ruled out. Wide surgical resection of the pedicle and base of tumor implantation may influence tumor recurrence, although further studies are needed.

Cardiac myxoma: single tertiary centre experience.

BACKGROUND: Although cardiac myxoma (CM) are rare and benign, they can cause life-threatening complications, such as hemodynamic disturbances or embolization. Surgical excision of the tumour is the treatment of choice. The aim of the study was to evaluate the epidemiological characteristics, clinical presentation, imaging findings, and outcomes of surgical treatment of patients with CM treated in the largest tertiary care centre in Slovenia. PATIENTS AND METHODS: We retrospectively analysed the medical records of all patients referred to our institution between January 2005 and December 2020 and identified 39 consecutive adult patients with pathologically confirmed CM. RESULTS: The average annual incidence of CM in the study was 3 per 2 million population per year. Patients were more often female (n = 25, 64%). The mean age at diagnosis was 63.1 ± 13.6 years. Dyspnoea was the most common presenting symptom (31%). CM was an incidental finding in 11 patients (28%). Seven patients presented with thromboembolic event (18%). Transthoracic echocardiography (TTE) was performed in all patients, however additional imaging was required in 22 patients (56%). All patients in our series were successfully treated surgically without in-hospital mortality. During the follow-up period (6 months to 16 years) three patients (8%) died, and all deaths were unrelated to CM. There was no recurrence of CM during the follow-up. CONCLUSIONS: Our single-centre study confirms that CM is rare cardiac tumour with diverse clinical presentation. Our data shows data that CM might be more prevalent than considered before. Surgical resection of the tumour is safe with excellent short- and long-term outcomes.

Cardiac Myxoma among Patients Undergoing Cardiac Surgery in a Tertiary Care Center: A Descriptive Cross-sectional Study.

INTRODUCTION: Heart neoplasms are rare tumors. Myxoma is the commonest primary benign tumor of the heart presenting with features of obstruction, arrhythmia, and embolism. Surgical excision of the tumor is the gold standard of treatment. The aim of the study is to find out the prevalence of cardiac myxoma among all cardiac surgeries operated during the study period. METHODS: A descriptive cross-sectional study was done among 3800 patients undergoing surgery for cardiac tumors in a tertiary care center after obtaining approval from the Institutional Review Committee (Reference number- 36/(6-11)E2/077/078). The data was collected retrospectively from August 2012 to August 2020 using convenience sampling method. Statistical analysis was performed using Microsoft Excel 2016. Point estimate at 95% Confidence Interval was calculated along with frequency, percentage, mean and standard deviation. RESULTS: There were 26 (0.68%) (0.42-0.94 at 95% Confidence Interval) myxoma among 3800 cardiac surgeries performed over eight years. The mean age of the patients was 54.76±14.31 (range 17-75) years. Twenty (76.92%) patients were females. The commonest presenting symptom was shortness of breath in 19 (73.07%) patients. En masse excision with the closure of the atrial septal defect was the principal surgical technique. The mean Intensive Care Unit stay and hospital stays were 2.92±1.29 and 6.26±2.61 days respectively. There was no perioperative mortality. CONCLUSIONS: Cardiac myxoma was the most common cardiac tumor encountered as in other studies.

Aortic valve myxoma-A systematic review of published cases.

BACKGROUND: Aortic valve myxoma is the rarest location of the most common primary tumour of cardiac origin. Because of the paucity of data, there is little known about their clinical presentation, diagnosis and complications. METHODS: PUBMED, EMBASE, SCOPUS and WEB OF SCIENCE were systematically searched to identify all published cases of aortic valve myxoma through October 2020. Descriptive statistics were used to report the data. RESULTS: Aortic valve myxomas were more prevalent in young (mean age 41 years) male (75%) patients. It most commonly involved the right coronary cusp (50%). Cerebrovascular events (25%), dyspnoea (18.8%), and distal embolisation (18.8%) were found to be the most frequent complications. Echocardiography remains the diagnostic modality of choice in all cases, histopathology is used for confirmation. Most cases were treated with surgical excision (94%); concomitant aortic valve repair and mechanical aortic valve replacement were performed in 25% and 37.5% cases respectively. Sudden cardiac death was noted in one patient. CONCLUSION: Aortic valve myxomas are more often than not discovered in the context of embolic phenomenon or dyspnoea. The most feared complication is stroke, although mortality remains low in surgically managed cases.

Clinical presentation of cardiac myxoma in a Singapore national cardiac centre.

INTRODUCTION: Cardiac myxoma is the most common cardiac tumour. In this study, we summarise our 17-year experience with the clinical presentation of cardiac myxoma at National Heart Centre Singapore, Singapore. METHODS: Between January 2000 and December 2016, retrospective data was reviewed for all consecutive patients who underwent surgical resection of cardiac myxoma. Patients' clinical characteristics were reviewed and described. RESULTS: A total of 67 (18 male, 49 female; mean age 53.1 ± 13.5 years) patients underwent cardiac myxoma resection. There were 19 (28.4%) patients with asymptomatic cardiac myxoma. There were no significant differences in gender; body habitus and myxoma size; and haemoglobin, white blood cell or platelet counts between patients with symptomatic and asymptomatic myxoma. However, the number of asymptomatic cardiac myxomas seemed to follow an increasing trend from 19.4% (period 2000-2008) to 36.1% (period 2009-2016), suggestive of an 'era effect'. CONCLUSION: In our study, a majority of patients were women, with a wide age range of 18-78 years. The diagnosis of asymptomatic cardiac myxoma was present in 28.4% of patients, with an increasing trend for incidence over the years. This is possibly due to increased opportunistic screening (with electrocardiography and clinical examination) as well as higher usage of medical imaging.

Predicting the risk of cardiac myxoma in Carney complex.

PURPOSE: Carney complex (CNC), is an autosomal dominant multiple neoplasia and lentiginosis syndrome. We aimed to identify risk factors associated with the occurrence and recurrence of cardiac myxomas, the predominant cause of death in CNC patients. METHODS: Patients with CNC were monitored prospectively between 1995 and 2020 for the development of cardiac myxomas. RESULTS: Of the 319 patients studied, 136 (42.6%) developed myxomas. The mean age at diagnosis was 28.7 ± 16.6 years in females and 25.0 ± 16.4 years in males. By age 30, 35% of females and 45% of males had at least one myxoma. The CNC-related lesions, lentigines, cutaneous, mucosal, or breast myxomas, thyroid nodules, pituitary adenoma, and schwannoma were significantly more frequent (all p < 0.05) among patients with myxomas. Forty-four percent of patients had recurrences; nearly all within the first 8 and 16 years for males and females, respectively. Recurrences were more common in females. CONCLUSION: This is the largest study to date and provides the first-time risk estimates by age and gender for cardiac myxomas in CNC patients. Cardiac myxomas are common by age 30 and often recur, especially in women, but the risk drops in 10 to 20 years. These findings may guide patient counseling, screening intervals, and surgical approaches. CLINICAL TRIAL REGISTRATION: Clinical Trial Registration: Defining the Genetic Basis for the Development of Primary Pigmented Nodular Adrenocortical Disease and the Carney complex, Registration number: NCT00001452 URL: https://clinicaltrials.gov/ct2/show/NCT00001452.

Epidemiología del mixoma cardiaco en una población española. Una serie quirúrgica de 30 años / Epidemiology of cardiac myxoma in a Spanish population. A 30-year surgical series

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[Clinical and pathological characteristics of cardiac tumors: analyses of 689 cases at a single medical center].

Objective: To study the clinicopathological characteristics of cardiac neoplasms. Methods: A total of 689 cases of cardiac neoplasms from January 1st 1992 to December 31th 2017 at Guangdong Provincial People's Hospital were collected. The clinical data and histologic features were analyzed along with a review of literature. The pathological diagnosis and classification were based on the criteria of WHO 4th edition(2015). Results: Among 689 cases of cardiac neoplasms, 259 were male and 430 were female patients, with age from 0 to 84 years (mean of 48 years). The peak incidence was between the fourth and sixth decade. Among patients younger than 20 years, there were 24 males and 12 females. 674 cases(674/689,97.8%)were primary cardiac tumors and 15 cases were secondary tumors (15/689,2.2%). Amongst the primary cardiac neoplasms, 625 cases were benign(625/674,92.7%), 7 cases were borderline (7/674, 1.0%), and 42 cases were malignant (42/674, 6.2%). The incidences of benign, borderline and malignancy heart tumors among patients below 20 years old were lower than those of patients over 20 years of age (4.8% vs. 95.2%; 3/9 vs. 6/9; 5.5% vs. 94.5%, respectively). Of the benign tumors, 406 cases were female and 219 cases were male. More male than female patients were seen in borderline and malignancy cardiac tumor categories (6∶3; 34∶21). Of 625 benign tumors, 577 cases were myxoma(85.6%), which mainly occurred in patients over 20 years of age(85.9% vs. 14.1%) with a female predominance. Non-myxomas mainly occurred in children and adolescent patients compared to adult (55.6% vs. 44.4%, P<0.01) with a male predominance. Overall, 524 tumors originated from the left atrium, 84 cases from the right atrium, 26 cases from the pericardium, 23 cases from the right ventricular, and 11 cases from the left ventricle. However, 21 cases were multicentric or involving cardiac valves. Benign tumors mainly involved left heart(76.3%) vs. right heart(81/625, 12.6%). The mostly common location of borderline tumors was right heart(5/9). Malignant tumors tended to involve the right heart(22/55,40.0%) and pericardium(18/55, 32.7%). Conclusions: Although the incidence of cardiac neoplasms is low,various tumor types can occur, most of which are myxoma with a female predominance. Non-myxomas mainly occur in children and adolescents with a male predominance.

Prevalence and Clinical Features of Mazabraud Syndrome: A Multicenter European Study.

BACKGROUND: Mazabraud syndrome is a rare disorder, characterized by the presence of fibrous dysplasia (FD) with associated intramuscular myxomas. Data are scarce on the prevalence, clinical features, and natural history of this disorder and outcomes. In this multicenter study, we evaluated a series of patients from 6 European centers. METHODS: All centers affiliated with the European Musculo-Skeletal Oncology Society (EMSOS) were invited to include data on all patients with Mazabraud syndrome who were seen between 1980 and 2015. The study investigated the prevalence of Mazabraud syndrome, the type, severity, and localization of FD lesions in relation to myxomas, the histopathology of myxomas, and results of GNAS-mutation analysis, when available. RESULTS: Thirty-two patients (22 female) from 6 centers were included. The prevalence of Mazabraud syndrome was 2.2% in the combined cohort of 1,446 patients with FD, and the syndrome was diagnosed at a mean of 10.1 years after diagnosis of FD. The myxomas were predominantly localized in the upper leg. Excision was performed in 20 patients, recurrence occurred in 6 of these patients (30%) at a median of 8.5 years (range, 1.9 to 16.0 years), and revision surgery was necessary in 5 (25%). High cellularity of myxomas was associated with recurrence (p < 0.05). A GNAS mutation was identified in the myxoma tissue of 5 (83%) of 6 patients with GNAS-mutation analysis. CONCLUSIONS: This study is the first, to our knowledge, to provide data on the prevalence of Mazabraud syndrome in a relatively large cohort. Although the outcomes of surgical resection were good, a quarter of the patients required revision surgery despite clear resection margins. High cellularity of myxomas was associated with recurrence. GNAS mutations were identified in 83% (5 of 6), emphasizing the shared origin of FD and myxomas. Our data show that patients with FD who have disproportionate complaints, irrespective of FD type, extent, or severity, should be investigated for the possible presence of myxomas. LEVEL OF EVIDENCE: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Differentiated thyroid cancer: Why does it affect predominantly women during the reproductive period and have higher incidence of mutual association with breast cancer?

Differentiated thyroid cancer (DTC) is markedly more common in women than men, and its occurrence and risk for poorer prognosis are associated with pregnancy. Further, it is known that there is a high frequency of co-occurrence of DTC and breast cancer. Although the underlying mechanisms that contribute to these phenomena are not entirely clear, 2 hypotheses are proposed here. First, human chorionic gonadotropin (hCG) produced by the placenta may be involved, since hCG has a similar function to stimulate the thyroid as thyroid-stimulating hormone (TSH), the latter of which is known to play a role in causing DTC and may promote breast cancer through the secretion of thyroid hormones (THs). Second, thyrotropin-releasing hormone (TRH), which is stimulated by suckling in the puerperal period, induces the secretion of not only TSH and thus indirectly THs, but also prolactin (PRL), which can accelerate the development of breast cancer. These hypotheses also explain the pregnancy-associated transient increase in breast cancer risk, while inhibition of estrogen by PRL may have a long-term preventive effect on breast cancer. Pregnancy-associated hyperthyroidism may also account for female preponderance of thyroid disease in general as well as tumors in organs that the thyroid hormone targets such as cardiac myxoma and diffuse-type gastric carcinoma.

Outcomes of Patients with Newly Diagnosed Cardiac Myxoma: A Retrospective Multicentric Study.

The patient database at the First Department of Internal Medicine in Martin, the Central Slovak Institute for Cardiac and Vascular Diseases in Banska Bystrica, and the National Slovak Institute of Cardiovascular Diseases in Bratislava was searched to identify patients with benign tumors of the heart seen during the 5-year period between 2011 and 2016. Forty-one patients with primary cardiac myxomas were identified and their medical records were reviewed for details pertaining to presenting symptoms, staging modalities, treatment approaches, and outcomes. Most of the studied patients were diagnosed with echocardiography (n = 35, 85%). The occurrence of the tumor was higher in the female population (n = 25, 61%). The most common presenting symptoms were dyspnoea (n = 17, 42%), chest pain (n = 3, 7%), or pain and paraesthesia of the limbs (n = 2, 5%). Acute embolic event due to embolization of tumor fragments resulted in cerebral stroke (n = 5, 12%). All patients were treated by resection. Only one comorbid patient died due to multiple-organ dysfunction syndrome two weeks after the resection. The most common postoperative complication was bleeding (n = 2, 5%) and infection (n = 2, 5%). The early diagnosis and appropriate treatment are often curative, with very low risk of recurrence. Postoperative survival is high.

Immunohistochemical characterization of glandular elements in glandular cardiac myxoma: Study of six cases.

Back ground: Glandular cardiac myxoma has varying clinical presentation with uncertain histogenesis and debatable immunohistochemical profile. Glandular epithelial differentiations are rare phenomenon known to be present as an intrinsic component of the tumor. The origin of the glands has been attributed to epithelial differentiation of a totipotent cardiomyogenic precursor cells or the entrapped foregut rests in the tumor. MATERIALS AND METHODS: Retrospective study includes six cases of glandular cardiac myxoma collected over a perior of 4 years. Sections were examined to define the histogenesis, histological and immunohistochemical profile of the glandular elements. RESULTS: Incidence of glandular cardiac myxoma was 6.6% with a male to female ratio of 1:2.Mean age was 49.9 years. Left atrium was the commonest site. Five were sporadic and one was familial. Chest pain and dyspnea were the commonest clinical symptoms. Histologically all myxoma showed well formed glandular structures with typical myxomatous area. No atypia, mitosis or necrosis was identified in the glandular elements. Markers in six cases of glandular cardiac myxoma were immunopositive for CK7, CK 19, EMA, CEA, focally for E-cadherin while immunonegative for CK20, Chromogranin, Synaptophysin, calretenin, vimentin, B-catenin, TTF-1 and GCDFP-15 favoring enteric differentiation. CONCLUSION: Glandular cardiac myxoma is a rare entity which shows characteristics similar to those of classical cardiac myxoma with benign glandular elements showing enteric differentiation. Complete surgical excision is the treatment of choice with good prognosis. It is important to recognize this entity to avoid an erroneous diagnosis of metastatic adenocarcinoma.

Identification and clinical course of 166 pediatric cardiac tumors.

The aim of this study was to investigate the pathological classifications, clinical features, and natural history of pediatric cardiac tumors to provide a basis for the selection of an appropriate therapeutic method. The medical records of in- or outpatients with cardiac tumors at four hospitals were classified to analyze various types of tumor growth locations, clinical manifestations, surgical indications, and long-term follow-up results. There were 166 patients, including 158 with primary cardiac tumors, six with metastatic cardiac tumors, and two with unclassified cardiac tumors. Among the 158 cases of primary cardiac tumor, 150 were benign and eight were malignant. The rhabdomyoma, fibroma, and myxoma are the most common types of benign cardiac tumors. The major clinical manifestations of cardiac tumors include outflow tract obstruction, arrhythmia, dyspnea, pericardial effusion, heart failure, and seizures. Among the 59 patients who underwent surgery, 49 had primary benign cardiac tumors, eight had primary malignant tumors, and two had malignant metastatic tumors. Post-surgery, nine of the patients had residual tumor tissues that did not significantly affect their hemodynamics. Following surgery, there were two cases of recurrence and nine deaths, including four of benign and five of malignant tumors with mortality rates of 8.2 and 50.0 %, respectively. Of the remaining 107 cases of patients who did not undergo surgery, five (4.7 %) died. CONCLUSION: The primary benign cardiac tumors are the predominant pediatric cardiac tumors, of which rhabdomyoma, fibroma, and myxoma are the most common types. If severe symptoms are nonexistent and the hemodynamics is unaffected, most of the patients can survive in the long term despite the tumors. What is known: • Pediatric cardiac tumors are rare and are predominantly primary and benign. • The symptoms of heart failure, arrhythmia, and outflow obstruction are the most severe complications of cardiac tumors. What is new: • The rhabdomyoma, fibroma, and myxoma are the most common types of primary benign cardiac tumors. • If severe symptoms are not present and the hemodynamics are unaffected, most of the patients can survive in the long term despite the tumors.

Cardiac masses : Experience from a Turkish tertiary center of cardiology.

OBJECTIVE: Cardiac masses comprise a category that includes benign, malignant, and nontumoral mass lesions. The present study aims to share the findings relating to cardiac masses observed at our tertiary cardiology hospital in Turkey. METHODS: The records of patients who presented with cardiac masses and underwent surgery at our institute between 2006 and 2015, and whose tissue samples were sent to a pathology laboratory, were retrospectively reviewed in a consecutive manner. RESULTS: The study included 228 patients with an average age of 52.5 ± 17.3 years. Of the study's subjects, 95 (41.7%) were male and 133 (58.3%) female. The most commonly observed mass was myxoma in 68 patients (29.8%), of whom 20 (29.4%) were male and 48 (70.6%) female. The second most frequently detected mass was pannus, with 38 cases (16.7%) - 10 (26.3%) in males and 28 (73.7%) in females. The third most common cardiac mass was thrombus (16.2%), with 18 cases of thrombi in men (48.6%) and 19 (51.4%) in women. CONCLUSION: The most commonly observed cardiac mass was myxoma and most were localized in the left atrium. The second most frequently detected mass was pannus, which was mostly found on mitral mechanical prosthetic valves. Thrombi were the third most prevalent mass and were commonly localized in the right atrium.

Mixoma ventricular izquierdo en paciente con síndrome de inmunodeficiencia humana / Myxoma left ventricle in a patient with human immunodeficiency syndrome

Fundamento: las complicaciones cardiovasculares son frecuentes en etapas finales del síndrome de inmunodeficiencia adquirida; los tumores cardíacos pueden observarse aunque con poca frecuencia.Objetivo: presentar el caso de una paciente de 30 años de edad, femenina y con antecedente de síndrome de inmunodeficiencia adquirida hace cuatro años y signos de insuficiencia cardíaca.Caso clínico: paciente de raza negra, femenina de 30 años de edad, que llevaba cuatro años con tratamiento antirretroviral para síndrome de inmunodeficiencia adquirida. Comenzó a presentar astenia marcada, disnea de moderada intensidad que fue aumentando y le impedía el decúbito supino, palpitaciones y edemas en miembros inferiores. Por esta sintomatología acude al servicio de urgencia.Conclusiones: la ecocardiografía es una herramienta valiosa a la hora de diagnosticar de forma rápida y sencilla la presencia de tumores cardíacos. Se debe sospechar la presencia de tumores cardíacos en pacientes con síndrome de inmunodeficiencia humana y signos de descompensación cardiaca. El diagnóstico definitivo del tipo de tumor de esta paciente, se realizó postmorten, ya que la paciente falleció por otras complicaciones clínicas.(AU)

Background: cardiovascular complications are frequent at final stages of acquired immunodeficiency syndrome. At these points, cardiac tumors can be seen with low frequency.Objective: to present the case of a patient with cardiac insufficiency signs and preceding four-year acquired immunodeficiency syndrome.Clinical case: a thirty-year-old black female patient, who received four-year antiretroviral therapy for acquired immunodeficiency syndrome. She began having palpitations, edemas of inferior limbs, noticeable asthenia, and low intensity dyspnea, which was increasing later. Because of this symptomatology, the patient goes to casualty department.Conclusions: echocardiography is a valuable tool when diagnosing the presence of cardiac tumors in a fast and simple way. Patients who suffer from acquired immunodeficiency syndrome and have cardiac decompensation signs are exposed to these types of tumors. A definitive diagnosis in this patient was made after her death (post-mortem), because she died due to other clinical complications.(AU)

Cerebral Metastases in Patients with Left Atrial Myxoma.

We describe two cases of cardiac myxomas with cerebral metastases and review the literature on this subject. doi: 10.1111/jocs.12727 (J Card Surg 2016;31:289-293).