Benign Cardiac Tumors Research Articles

New insights into the pathogenesis of cardiac papillary fibroelastomas.

Cardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors typically found on the heart valves. The previously published data on the CPF focused on its clinical presentation, optimal management, and prognosis. However, histogenesis of these lesions remains controversial. Accordingly, the aim of this study was to establish the role of endocardial endothelium (EE) in CPF formation. Four CPF tumors removed from the right atrioventricular valves were analyzed using hematoxylin & eosin, orcein, and Masson trichrome staining together with immunochemistry for CD-34, CD-68, vimentin, vWF and a-SMA. Moreover, conventional transmission electron microscopy was used for morphological analysis and a-SMA presence confirmation. Ultrastructural morphology, immunohisto- and immunocytochemical analyses indicated that cells covering collagenous core have an endothelial origin. Some endocardial endothelium cells have the potential to undergo a transition to mesenchymal cells. Moreover, the abundant presence of extracellular vesicles may indicate an active intercellular communication. Within the intermediate translucent zone, amorphous substances with monocytes/macrophage-like cells and fibroblastic cells were found. Finally, within collagenous core activated (myo)fibroblasts were observed. Our study demonstrated that the endocardial endothelium of the CPF was "double-sided", i.e., it presented both endothelial and mesenchymal cell characteristics. Another finding was the presence of monocytes, and macrophages which were integrated into CPF core and displayed features of a fibroblast that have been shown to contribute to extracellular matrix production. This could be interpreted as being attributed to the CPF histogenesis.

413 Challenges in the field of cardiac tumours: the surgical experience of Trieste

Abstract Aims Cardiac tumors are rare and heterogeneous entities which still remain a diagnostic and therapeutic challenge. The treatment for most cardiac tumors is prompt surgical resection. We sought to provide an overview of surgical results from a series of consecutive patients treated at our tertiary centre during almost a 20-year experience. Methods and results In this single centre study, 55 consecutive patients with cardiac tumor underwent surgical treatment from January 2002 to April 2021. Of these, 42% of patients were male and the mean age was 62 ± 12 years. Almost 27% of patients were symptomatic at the time of the diagnosis, mostly for dyspnoea and palpitations. The most frequent benign cardiac tumor was myxoma (58% of cases), occurring mainly in the left atrium (97%). Pleomorphic sarcoma was the most frequent primary malignant cardiac tumour (7.2% of cases), mainly located in the ventricles (25% left ventricle; 50% right ventricle). In all cases of benign tumors surgery was successful with no relapses. In 50% of cases of pleomorphic sarcoma relapses were observed during follow-up. After a median follow-up of 44 months, 15 (27%) patients died. While malignant tumors presented a limited survival, benign tumours showed a very good prognosis. Conclusions Cardiac tumours require a multidisciplinary work-up to guarantee a prompt diagnosis and appropriate treatment. In our surgical experience, the prognosis of benign tumours was excellent, while malignant tumours had poor outcomes despite radical surgery.

An Uncommon Cause of Dyspnea: Usefulness of Multimodality Cardiac Imaging

Pericardial lipomas are very uncommon benign primary cardiac tumors. We describe the case of a patient with symptomatic large pericardial mass who presented with heart failure. Multimodality cardiac imaging helped us in the diagnosis of this unusual entity. (Level of Difficulty: Intermediate.)

Diagnostic mystery—a rare right ventricular cardiac hemangioma: a case report

BackgroundCardiac hemangiomas are rare in all kinds of benign cardiac tumors. Although cardiac hemangiomas affect all ages and may occur anywhere within the heart, right ventricular hemangiomas are extremely uncommon.Case presentationWe report a 56-year-old woman presented with chest tightness and breath shortness for 3 months. Transthoracic echocardiography and coronary computed tomography angiography showed a mass located adjacent to the apex of the right ventricle but both failed to figure out where the mass originated from, remaining a diagnostic mystery preoperatively. The mass was removed successfully and the histopathological examination confirmed it was hemangioma.ConclusionsCardiac magnetic resonance should be the ultimate diagnostic tool of cardiac tumors. Surgical removal, associated with a low recurrence rate and long-term survival benefits, should be the first choice of therapy for cardiac hemangiomas.

C76. Primary Cardiac Tumors : Benign or Malignant ? The Importance of Clinicopathologic Confirmation : a case series

Abstract Background Cardiac tumor is a rare condition of space-occupying lesion mass in cardiac chamber. The incidence of clinically diagnosed Primary Cardiac Tumors is approximately 1,380/100 million individuals. Majority of cases are benign, with nearly half of them are cardiac myxoma. Although early clinical symptom of benign and malignant cardiac tumor are similar, the management and prognosis are much different. Objective To present serial cases of benign and malignant primary cardiac tumor. Case Description Mrs. RH, a 60-year-old woman referred to heart failure clinic National Cardiovascular Center Harapan Kita with chief complaint of short of breath on exercise. Echocardiography confirmed a huge mass obstructed her left atrium. She was hospitalized urgently with giant Left Atrial Myxoma with normal coronary artery, then underwent surgery of LA myxoma extirpation, Mitral Valve repair and Tricuspid Valve repair. Histopathological analysis confirmed of LA myxoma. Mr. RBM, a 42-years-old man came to emergency unit at National Cardiovascular Center Harapan Kita with chief complaint shortness of breath and palpitation in about 1 month. A history of a cardiac tumor extirpation and mitral valve replacement was done in Penang Adventist hospital 10 months before. Transthoracal and transesophageal echocardiography found solid mass obstructing mechanical valve. Histopathology analysis showed undifferentiated cardiac sarcoma and against LA myxoma. Discussion Clinical course and management of cardiac tumor depends on early detection and histopathologic analysis. Although surgery is the cornerstone for cardiac tumor management, but confirmation of histopathologic findings is important.

C76. Primary Cardiac Tumors : Benign or Malignant ? The Importance of Clinicopathologic Confirmation : a case series

Abstract Background Cardiac tumor is a rare condition of space-occupying lesion mass in cardiac chamber. The incidence of clinically diagnosed Primary Cardiac Tumors is approximately 1,380/100 million individuals. Majority of cases are benign, with nearly half of them are cardiac myxoma. Although early clinical symptom of benign and malignant cardiac tumor are similar, the management and prognosis are much different. Objective To present serial cases of benign and malignant primary cardiac tumor. Case Description Mrs. RH, a 60-year-old woman referred to heart failure clinic National Cardiovascular Center Harapan Kita with chief complaint of short of breath on exercise. Echocardiography confirmed a huge mass obstructed her left atrium. She was hospitalized urgently with giant Left Atrial Myxoma with normal coronary artery, then underwent surgery of LA myxoma extirpation, Mitral Valve repair and Tricuspid Valve repair. Histopathological analysis confirmed of LA myxoma. Mr. RBM, a 42-years-old man came to emergency unit at National Cardiovascular Center Harapan Kita with chief complaint shortness of breath and palpitation in about 1 month. A history of a cardiac tumor extirpation and mitral valve replacement was done in Penang Adventist hospital 10 months before. Transthoracal and transesophageal echocardiography found solid mass obstructing mechanical valve. Histopathology analysis showed undifferentiated cardiac sarcoma and against LA myxoma. Discussion Clinical course and management of cardiac tumor depends on early detection and histopathologic analysis. Although surgery is the cornerstone for cardiac tumor management, but confirmation of histopathologic findings is important.

Usefulness of CHA2DS2-VASc scoring system for predicting risk of embolism in patients with cardiac tumours: a single-centre study

Abstract Background The CHA2DS2-VASc score is the most used in predicting thromboembolic risk in patients with atrial fibrillation (AF). However, a higher CHA2DS2-VASc score predicts cardiovascular events even in patients without AF. Up to date, there is limited evidence about the association between CHA2DS2-VASc score and the embolic risk in patient with cardiac tumours (CT). Purpose To evaluate the role of the CHA2DS2-VASc score in predicting embolism and to identify other markers of embolization in patients with CT. Methods We included all patients with a CT who had a complete echocardiographic evaluation and a histologic diagnosis. We excluded patients with cardiac thrombi because of their higher embolic risk. Results Our sample consisted of 221 patients (mean age 60.9±15.6, 7% female). 132 (59.7%) patients had benign CT, usually left-sided (84.8%). In the malignant tumours, 28 (31.5%) were primary and 61 (68.5%) were metastasis. Compared to benign tumours, malignant ones were predominantly right-sided (47.2%) or with pericardial (20.2%) or great-vessels involvement (16.9%). A total of 59 patients (26.6%) experienced an embolic event, of which 33 patients had peripheral embolism, while 28 had pulmonary embolism (PE), 2 patients had both. Patients with embolism were older (p=0.013), had a higher prevalence of diabetes or previous stroke (p=0.019 and p<0.001), had left-sided and not-infiltrating CT (p=0.001 and p=0.04). We did not find any differences in AF prevalence, atrium volumes or other CT characteristics. Moreover, patients who developed an embolic event had a higher CHA2DS2-VASc score than those without embolism (p<0.001). In particular, a CHA2DS2-VASc score >3 had an AUC=0.835 in predicting a peripheral embolic event in the overall population (p<0.001). At multivariate analysis, only CHA2DS2-VASc and left-side localization were significantly associated with embolization (p<0.001 and p=0.009). Also in patients with right-sided CT, CHA2DS2-VASc score (p=0.017), together with some tumours characteristics (mobility, pericardial effusion and absence of myocardial infiltration (p<0.04 for all) was associated with PE. Embolization did not impact survival when considering the overall population. Otherwise, when focusing on patients with malignant CT, those who had an embolic event had a worse prognosis (p=0.02), as well as those with PE (p=0.037). Conclusions CHA2DS2-VASc and left-side localization are the best markers of embolism. In particular, CHA2DS2-VASc seems to predict embolization in CT, regardless of histology or localization. Many patients with CT and embolism may not be offered surgical treatment given their assumed high-risk profile. By contrast, our analysis showed that survival is not related to the embolic event per se, but by histology. In patients with CT and high CHA2DS2-VASc score, further studies are needed to evaluate the best therapeutic strategy to minimize the embolic risk. Funding Acknowledgement Type of funding sources: None.

A Rare Reason of Cardiogenic Shock and Acute Myocardial Infarction: Aortic Valve Myxoma

Aortic valve myxoma is a rare benign cardiac neoplasm. The association of aortic valve myxoma with cardiogenic shock and acute myocardial infarction has been reported in few observations. We report the case of a 19-year-old male patient, who underwent chest pain for two weeks, then further examinations indicated a soft spherical mass on the left coronary cusp. The patient had sporadic cardiogenic shock and acute myocardial infarction during the preoperative preparation, and we carried out emergency effective cardiopulmonary resuscitation (CPR), followed by emergency surgical operation for aortic valve tumor. Postoperative pathology showed it was a myxoma. The patient recovered smoothly and was discharged on postoperative day 7. Cardiogenic shock and acute myocardial infarction are very nonspecific, and we should be aware that patients with cardiogenic shock and acute myocardial infarction possibly suffer from aortic valve myxoma.

Assessment of cardiac tumors by 18F-FDG PET/CT imaging: Histological correlation and clinical outcomes

BackgroundTo evaluate the diagnostic value of 18F-FDG PET/CT in distinguishing benign versus malignant cardiac tumors as well as to assess its prognostic value. MethodsWe analyzed 38 patients with cardiac tumors who underwent 18F-FDG PET/CT and followed for median 8.5 ± 12.5 months. SUVmax and TBRmax (maximum tumor-to-background ratio) by receiver-operating characteristic (ROC) curve analysis were used to obtain threshold for the diagnosis of malignancy as defined by histology (n = 38). Survival was assessed and correlated with the dignity of the lesions and PET parameters. ResultsOptimal cut-off values indicating malignancy were as follows: SUVmax = 3.44, with 100% sensitivity and 92.9% specificity, and TBRmax = 1.55, with 95.8% sensitivity and 92.9% specificity. A significant difference of 18F-FDG uptake was observed between primary benign (n = 14, SUVmax = 2.35 ± 1.31, TBRmax = 1.05 ± 0.50) compared to primary malignant cardiac tumors (n = 11, SUVmax = 8.90 ± 4.23, TBRmax = 3.82 ± 1.44) as well as cardiac metastases and lymphoma (n = 13, SUVmax = 14.37 ± 8.05, TBRmax = 6.19 ± 3.38) (all P < .001). Survival rate was significantly lower in patients with malignant as compared to benign cardiac tumors (P < .05). Regression analysis revealed that the lesion dignity determined by the cut-off value of SUVmax was an independent predictor for death in patients with cardiac tumors (P < .05). Conclusion18F-FDG uptake in cardiac tumors can differentiate between benign and malignant cardiac tumors and predicts survival.

Cardiac myxoma as a potential trigger of takotsubo cardiomyopathy: A brief review on mechanistic and clinical perspectives.

In clinical practice, cardiac myxomas constitute the majority of benign cardiac neoplasms, and might potentially present with a variety of embolic, obstructive as well as constitutional symptoms. On the other hand, these neoplasms might be potentially associated with the evolution of takotsubo cardiomyopathy (TTC) that is universally considered as a transient form of acute myocardial dysfunction. Accordingly, the present paper primarily aims to focus on potential mechanisms and associated clinical implications of TTC evolution in the setting of cardiac myxomas.

Neonatal infective endocarditis in a cardiac rhabdomyoma case: A rare presentation

Neonatal infective endocarditis (IE) is an exceedingly rare disease and usually not associated with cardiac rhabdomyomas or any underlying structural cardiac anomalies. Cardiac rhabdomyoma is also the most common benign primary cardiac tumor. The prognosis depends on the size, location of tumors, and outflow tract obstruction but can regress within 2 months of age and reduces the necessity of surgery. Due to the variable clinical features and course, we need to evaluate cardiac vegetation as soon as possible for better outcomes. A combination of these two conditions was not reported before. Here, we presented a case of IE with cardiac rhabdomyoma in a male baby which is first reported from Bangladesh previously.

Benign Cardiac Neoplasms in the United States: A Thirteen-Year Review

Cardiac neoplasms are uncommon tumors. For epidemiological purposes, they can be divided into benign and malignant subtypes, with the former occurring at a significantly higher rate than the latter. Due to their uncommon nature, there are few data-driven studies examining the characteristics and trends of benign cardiac neoplasms. Our retrospective HCUP-NIS data review purports to illuminate some of the trends surrounding benign cardiac neoplasms and their associated co-occurrences. The data consisted of 482,872,274 weighted discharges. There were 45,568 weighted discharges that included a benign cardiac neoplasm. Benign cardiac neoplasms were more often observed in women (64.33%), and the average age was 63.8 years. The most common cardiovascular co-occurrences in patients with benign cardiac neoplasm were atrial tachyarrhythmias (28.93%), heart failure (19.61%), and embolic events such as stroke, myocardial infarct, or pulmonary embolism (19.82%). Other co-occurrences included pulmonary hypertension (7.55%), ventricular arrhythmias (3.23%), and other EKG abnormalities (3.70%). Procedures were numerous in patients with benign cardiac neoplasms. 43% of patients with this diagnosis had some form of cardiac surgery during their hospitalization. Overall, this study found low incidence of benign cardiac neoplasms in the USA during this 13-year study period. However, in the presence of benign cardiac neoplasms, our study showed that cardiovascular co-occurrences are not uncommon and may help to illuminate this otherwise rare diagnosis.

Abstract P172: Pulmonary Valve Papillary Fibroelastoma In A Patient With Chronic Chest Pain

Primary cardiac tumors are rare entity, and the most common benign cardiac neoplasms include myxomas, lipomas and papillary fibroelastomas (PFE) with PFE accounting for 8% of the tumors. A 53-year-old female presented with chronic chest pain and exertional shortness of breath. She first sought evaluation four years ago, when her pulmonologist referred her to thoracic surgery after an incidental finding of a 3.9 cm anterior mediastinal mass on CT-scan (Figure 1A-B). The patient described the pain as a constant pressure and squeezing sensation in her chest and worsening shortness of breath while laying on her sides, relieved only when a pillow was propped up under her chest. The pain did not significantly reduce her mobility or capacity to work. Initial evaluation, including transthoracic echocardiogram, stress testing, carotid ultrasound, and pulmonary function testing - was performed and found to be unremarkable. Further evaluations revealed a hiatal hernia, leading her symptoms to be attributed to GERD as causing her persistent non-exertional chest pain. The patient remained in good health despite her symptoms persisting. She later re-sought attention for chest pain on exertion and hoarseness three years later. She again underwent a full cardiopulmonary workup, yielding benign findings on EKG, troponins, and echocardiogram. Due to no alarming symptoms at the time, she was subsequently discharged. The patient continued to endorse chest pain and a follow-up MRI revealed a now 5 cm anterior mediastinal mass, thought to be a thymic cyst. Over the next few months, the patient continued to endorse worsening chest pain and exertional shortness of breath. She followed up with her cardiologist and a subsequent TEE was significant for a pulmonic valvular mass. She was admitted to the hospital for further investigation. During her hospital stay, the patient underwent surgery to resect the anterior mediastinal mass likely to be a thymic cyst and the pulmonary artery mass which was determined to be a pulmonary valve papillary fibroelastoma in the histopathology exam (Figure 2). The surgery was successful with no complications postoperatively. The patient was discharged with no further chest pain and with improvement of the exertional shortness of breath.

Caseous calcification of the mitral annulus mimicking benign cardiac tumour of the mitral valve

Caseous calcification of the mitral annulus (CCMA) is a rare subtype of mitral annular calcification. It usually appears as a large, round, mass-like calcification with an echolucent core, which may be misdiagnosed as an intracardiac mass, cyst, thrombus or abscess of the mitral valve. We present a case report of CCMA that was misdiagnosed by echocardiography as a benign tumour due to its atypical imaging. The mitral valve mass was resected and it was pathologically confirmed to be a myxoid change with calcification. Echocardiography is the preferred initial diagnostic tool. Myocardial contrast echocardiography (MCE) is used to evaluate the vascularity of intracardiac masses or mass-like lesions, but neither echocardiography nor MCE is reliable for identifying atypical lesions. Cardiac computed tomography is helpful in establishing a diagnosis by showing dense calcifications, while cardiac magnetic resonance imaging is used primarily as a credible tool. We therefore recommend that a diagnosis should be made based on various imaging modalities, if necessary, and operators should be skilled to avoid misdiagnosis.

Total endoscopic left ventricle lipoma removal

BackgroundLeft ventricle (LV) lipoma is a very rare, benign cardiac tumor. Due to its rarity, LV lipoma is often misdiagnosed. Aspecific symptoms such as murmurs, arrhythmias, memory loss and palpitation may occur due to the mass effect.Case presentationWe report a case report of a 42 year old woman who was found to have left ventricle mass after check-up for arrhytmia. By a fully endoscopic approach, the mass was successfully resected from the left ventricle without the need for sternotomy.ConclusionTotal endoscopic removal of left ventricle lipoma’s can be done safely and has several advantages to conventional sternotomy. Larger studies are needed to confirm this hypothesis.

Right Anterolateral Thoracotomy Versus Sternotomy for Resection of Benign Atrial Masses: A Systematic Review and Meta-Analysis.

Primary benign cardiac tumors are rare disease entity that predominantly originate from the atria. Benign masses can induce heart failure, arrhythmia, or thromboembolic events. Therefore, surgical excision is often indicated. Current guidelines on the preferred approaches for resection (i.e., median sternotomy [MST] or right anterolateral thoracotomy [RAT]) are lacking. The aim of the current meta-analysis was to evaluate all studies comparing RAT to MST for excision of benign atrial masses in terms of safety, efficacy, and complications. The PubMed and EMBASE databases were searched through 9 June 2020. Data regarding mortality, complications, recurrence, ICU stay, and length of hospital stay were extracted and submitted to meta-analysis using random effects modelling. Heterogeneity was assessed by the I 2 test. Four retrospective observational studies were included, including 196 patients (RAT n = 97, MST n = 99). Mortality was 0% in both groups. Recurrence was <1% in the RAT group and 0% in the MST group. Complication rate tended to be lower in favor of the RAT group. Furthermore, RAT was associated with lower length of ICU stay (-17.7 hr, P = 0.01) and hospital stay (-4.0 days, P < 0.001). No significant differences in cardiopulmonary bypass (P = 0.09) and cross-clamp times (P = 0.15) were observed. The RAT approach is as safe and effective as MST for the excision of benign atrial masses. Moreover, RAT is associated with a reduced complication rate and a reduced duration of hospitalization and could be considered as the preferred approach in anatomically suitable patients.

Surgical treatment of primary cardiac tumors in the contemporary era: A single-centre analysis.

BackgroundPrimary cardiac tumors (PCT) are rare lesions but have the potential to cause significant morbidity if not timely treated. We reviewed our single‐center experience in the surgical treatment of PCT with a focus on the long‐term outcome.MethodsFrom 2001 to 2020, 57 consecutive patients underwent surgical resection of PCT at our Institution. Data including the demographic characteristics, tumor histology, surgical procedure, and postoperative outcomes were collected and analyzed.ResultsMean age at presentation was 63.6 ± 11.2 years, and 33 (57.9%) of the patients were female. A total of 55 (96.5%) subjects were diagnosed with benign cardiac tumor, while the remaining had malignant tumors. The most common histopathological type was myxoma. All patients survived to hospital discharge. Main postoperative complications were: acute kidney injury (n = 3), sepsis (n = 3), and stroke (n = 2). Mean follow‐up time was 9 ± 5.9 years. Long‐term mortality was 22.8% (13/57). No tumor recurrence was observed among survivors. There was a significant relationship between mortality and pathological characteristics of the tumor, and myxomas had higher survival rates.ConclusionSurgical treatment of PCT is a safe and highly effective strategy associated with excellent short‐term outcomes. Long‐term survival remains poor for primary malignant tumors of the heart.

Giant Right Atrial Myxoma Associated With Thrombocytopaenia

Cardiac myxomas are predominantly benign primary cardiac tumours that most commonly occur in the left atrium, with the second most common location being the right atrium [1]. We present the unusual case of a 46-year-old woman with a giant cardiac myxoma seen on imaging in the right atrium or right ventricle throughout the cardiac cycle (Figure 1A and B, CT angiography; Supplemental Videos 1-2, video echocardiography) and thrombocytopaenia which resolved after tumour excision.

Complete surgical resection of giant fibroma of the interventricular septum and left ventricle in an infant.

Ventricular fibroma is the second most common benign cardiac tumor in children, yet responsible for significant morbidity and mortality. Surgical management of fibroma in infants and children is controversial, including resection1 (often partial1,2), single-ventricle palliation, or transplantation.3,4 We present a symptomatic infant with giant fibroma involving the interventricular septum (IVS) and the left ventricle (LV) who underwent complete surgical excision.

The Question of Relapse of a Cardiac Myxoma: an Unusual Case from Practice

Various studies demonstrate that recurrence of cardiac myxoma (CM) occurs in 2.1–5.3% of cases. In cases of treatment of familial myxoma, the need for reoperations can reach 20–25%. It should be taken into account that among all benign cardiac tumors, CM is detected in up to 80% of cases, and the need for its surgical removal is beyond doubt.&#x0D; The aim. To present a case that is of particular interest due to an unusual multiple myxomas lesion of the heart of multi-chamber localization.&#x0D; Material and methods. This article presents an unusual case of detecting multiple myxomas of multi-cameral heart lesions 6 years after removal of myxoma of the left atrium of typical localization. The distinguishing feature of the course of the disease lies in the fact that in this case, a careful study of the life history did not reveal the characteristic signs of familial myxomas and Carney complex which is most often characteristic of multi-chamber lesions of the heart by a tumor process. At the same time, during the surgical removal of the myxoma of the left ventricle, the myxomas of the left and right atrium were revealed, which began to grow. A technical feature of the removal of the left ventricular myxoma of the apical localization was the use of video cameras and instruments for thoracoscopic surgery, which greatly facilitated revi-sion and removal of the hard-to-reach neoplasm. Our experience in surgical treatment of CM equals to 868 operations. Wherein, repeated operations performed in 12 patients were due to recurrence or appearance of a tumor in place other than the primary localization.&#x0D; Conclusions. CM recurrence is one of infrequent complications in the postoperative period, which, however, requires some vigilance and annual Echo monitoring. In the vast majority of cases, CM was represented by the so-called sporadic myxomas, which are more often localized in the left atrium, and rarely recur. If CM is suspected, it is necessary to exclude the possibility of “myxoma syndrome”, which has the frequency of 0.8%, according to our data. The use of the instruments for thoracoscopic surgery can greatly facilitate the removal of hard-to-reach heart tumors localization.