Primary Cardiac Tumors Research Articles

A Rare Case of Surgical Treatment of Gastric Myxoma in a 2-Year-Old Child

The consistency of the myxoma is often dense fibrous, or jelly-like mucous. Myxoma consists of undifferentiated mesenchymal cells of various shapes (rounded, stellate), which are located in the myxoid stroma. Myxoma is the most common primary cardiac tumor (up to 50% of all primary cardiac neoplasms). Myxoma of the stomach is a rare pathology. We could not find any information covering the issues of gastric myxoma in children. This article provides a clinical observation of a good result of surgical treatment of gastric myxoma in a 2-year-old child. The described clinical case indicates the need to create a unified register of rare tumors for their study.

A Case of Left Atrial Myxoma Diagnosed on Routine Preoperative Evaluation

Cardiac tumors are very rare and myxoma is the most common primary cardiac tumor. Cardiac myxomas frequently originate from the left atrium and predominantly affect women. We present a case of a 65-year-old male who was admitted for hernia surgery and underwent preoperative evaluation. On evaluation, he was found to have a diastolic murmur on auscultation and had a history of exercise intolerance, and a transthoracic echocardiogram was done. It revealed a large mass in the left atrium attached to the interatrial septum suggestive of left atrial myxoma. He then underwent surgical resection of the tumor. The lack of recognition of this condition by appropriate history and physical exam can lead to a delay in diagnosis and treatment and the risk of cardiovascular complications.

Age and treatment disparities in survival of primary malignant cardiac tumors: an analysis of over 40 years and 500 patients.

Primary malignant cardiac tumors (PMCTs) are rare and tend to have a poor prognosis, due to their aggressive biological behavior and the inadequate expertise with the disease. This article compares the survival of patients with PMCT subtypes in the United States across age and treatment groups. Data of 529 patients diagnosed with PMCTs were analyzed. Chi-squared test was used to assess significance of the differences between proportions in demographic and tumor characteristics by age and treatment. Cox regression analysis was used to estimate survival from the Surveillance, Epidemiology, and End Results (SEER) follow-up data. Survival rates for PMCTs differed significantly between age groups, with patients younger than 20 years surviving significantly longer than those older than 80 years. The median survival times of all patients with PMCTs were 22.5, 11, 5, and 1 month for ages less than 20, 20-50, 51-80, and greater than 80 years, respectively (global log-rank P=0.0026). In the treatment cohort, for all tumors [hazard ratio (HR) 1.52, P<0.001], sarcomas (HR 1.83, P=0.002), and other tumors (HR 2.24, P=0.017), survival was lower in patients who did not receive treatment than in those who received only surgery. Survival after diagnosis of sarcoma was lower in patients who received radiotherapy only than in those who received surgery only (HR 1.49, P=0.046). However, there was no significant association between treatment and survival for lymphoma and mesothelioma. This study confirms that PMCTs have limited treatment options and poor patient survival, especially for elderly patients and patients who receive no treatment. And patients with PMCTs of any age, whether treated or not, have poor survival rates. Techniques for early diagnosis and treatment may be necessary. Surgical treatment should have a higher priority for future treatment of patients with sarcomas.

Principal Diagnosis and Independent Predictors for 30-Day Readmissions in Primary Cardiac Tumor Patients

Principal Diagnosis and Independent Predictors for 30-Day Readmissions in Primary Cardiac Tumor Patients

Multimodality Imaging of Cardiac Paragangliomas.

Paragangliomas are rare neuroendocrine tumors of extra-adrenal autonomic paraganglia origin. Paragangliomas rarely involve the heart, and they account for less than 1% of primary cardiac tumors. Most cardiac paragangliomas are incidentally detected at echocardiography or CT or during the workup of symptomatic patients with high catecholamine levels. Paragangliomas are typically located around the great vessels, coronary arteries (atrioventricular groove), or the atria, which can be explained by the tumor origin from the paraganglia and the distribution of the cardiac plexus. At MRI, cardiac paragangliomas typically have low to intermediate signal on T1-weighted images and high signal on T2-weighted images. The tumors are strongly vascularized, with high uptake on first-pass perfusion images and a heterogeneous pattern on late gadolinium enhancement images. Functional imaging is indicated for diagnostic confirmation and to screen for additional tumor locations or metastatic disease. Surgical excision is the only curative treatment. Cardiac CT angiography or invasive angiography should be performed preoperatively to precisely delineate tumor vascularization. In particular, its relation with the coronary arteries should be determined, as paragangliomas can be perfused by the coronary arteries, posing additional surgical challenges and the need for coronary revascularization. This imaging essay reviews the characteristics of paragangliomas and the use of multimodality imaging for diagnosis and treatment. Keywords: CT Angiography, Molecular Imaging, MR Imaging, PET/CT, Cardiac, Heart, Neoplasms-Primary © RSNA, 2023.

Surgical treatment of primary cardiac tumors in children

ObjectiveSummarizing the treatment experience of primary cardiac tumors in children.MethodsThe date of 24 children with primary cardiac tumors who underwent surgery in our department from July 2003 to September 2022 was collected and analyzed treatment efficacy.ResultsAll patients completed the surgery successfully, including 21 cases of complete tumor resection, 2 cases of partial tumor resection, and 1 case of tumor biopsy. The location: 5 cases in the right atrium, 5 cases in the right ventricle, 6 cases in the left atrium, 6 cases in the left ventricle, 1 case in the left, right ventricle and ventricular septum, and 1 case in the ventricular septum. 23 cases were benign: 11 cases of myxoma, 7 cases of fibroma, 3 cases of rhabdomyoma, 1 case of infantile capillary hemangioma, and 1 case of lipoma. There was 1 case of borderline or malignant tumor. 23 patients were discharged successfully, 1 patient died of cardiac failure on the first day after operation. Follow-up was done from 5 months to 19 years and 2 months, 2 cases were lost to follow-up, and 1 case died of cardiac failure in the second year after operation due to severe mitral regurgitation. There was 1 case of tumor biopsy with space-occupying lesion gradually shrinking during follow-up. The prognosis of another 19 children with complete or partial tumor resection was good. There was no recurrence, enlargement, or reoperation of the tumor during the follow-up period.ConclusionsPrimary cardiac tumors in children are mostly benign. Surgery is effective, but the timing of surgery depends on the patient's condition.

Intramyocardial choristoma-a rare entity: first-ever case report and literature review

We report the first-ever case of ‘Intramyocardial choristoma’ documented in English-language literature. A 71-year-old woman, during her preoperative echocardiogram assessment, noted to have a large left ventricle (LV) mass with severe mitral regurgitation (MR). The patient was asymptomatic, with CT scan reported a large dense calcified LV mass. She underwent mitral valve replacement with LV mass resection operation. The mass was completely excised. Histopathological examinations show intramyocardial choristoma, with the lesion shows cardiac muscle with areas of calcification and mature bone tissue, with clear margins. Discharged well post operatively. Commonest clinical scenarios for intracardiac masses are myxomas, infective endocarditis with vegetation and thrombus. Primary cardiac tumours are very rare. Choristoma are rare benign tumour, with histologically normal tissues in an abnormal location, with a very rare recurrence rate. Transthoracic echocardiogram is reliable for the evaluation of the intracardiac masses, but the use of contrast echocardiogram gives additional benefit in analysing the characteristics of the intracardiac mass. For complex lesion, such as intramural component, extension into the inflow or outflow and into pericardial/extracardiac, MRI have added benefits. The treatment of choice is complete surgical resection. Follow up surveillance scans are recommended, as there is a very low risk of recurrence with uncertain prognosis. A multidisciplinary team approach is highly recommended in managing the patient pre-operative, intra-operative and post-operatively. Post operatively, regular follow up with imaging is recommended.

Clinical characteristics of patients referred to cardio-oncology clinic

To explore characteristics of outpatients in a single cardio-oncology clinic, patients visiting cardio-oncology clinic of Fuwai Hospital CAMS&PUMC (Beijing, China) from January 2020 to December 2021 were analyzed retrospectively. In total, 330 patients were included, the median age (Q1, Q3) was 58(46, 66) years, and there were 192 females (58.2%). The purposes for visit included an evaluation and treatment of cardiovascular adverse reactions (n=247, 74.8%), pre-antitumor therapy assessment (n=51, 15.5%), and management of primary or metastatic cardiac tumors (n=32, 9.7%). For patients with cardiovascular adverse reactions, the most common tumor type was breast cancer (n=88, 29.5%), followed by gastrointestinal cancer (n=70, 23.5%), and hematological cancers (n=62, 20.8%). Among them, 236 cases (95.5%) had received antitumor drugs in the past; 38 cases (15.4%) had a history of chest radiotherapy; some cases were complicated with hypertension (n=69, 23.2%) and/or hyperlipidemia (n=69, 23.2%); 42 cases (14.1%) had a history of coronary heart disease; and 16 cases (5.4%) were complicated with atrial fibrillation or flutter. Among 32 patients with cardiac tumors, 11 cases (34.4%) had primary malignant tumors; 6 cases (18.8%) had benign tumors; 2 cases (6.3%) had metastatic tumors; and 13 (40.6%) had unknown pathological types. This study explores the epidemiology of cardio-oncology in China and provides clinical insights for the future development of cardio-oncology. In the future, it is still necessary to study the benefits of cardio-oncology clinics and develop standardized indicators to evaluate their benefits.

The characteristics of invasive cardiac lipoma: case report and literature review.

Invasive cardiac lipoma is a rare type of primary cardiac tumor that is composed of adipose tissue but infiltrating the adjacent structures. It is a benign tumor that can cause significant morbidity and mortality due to its size and location within the heart. We describe a giant invasive intracardiac lipoma across atrial wall extending to the ascending aorta and the superior vena cava. This review will provide an overview of invasive cardiac lipoma, including its clinical presentation, diagnosis, and management.

Case of left atrial myxoid sarcoma with lung metastases presented as a cardiogenic shock: a case report and literature review.

We are presenting a case of a 41-year-old female with left atrial myxoid sarcoma, which was presented with a cardiogenic shock picture. She underwent surgical excision of the mass and was discharged in good condition. After discharge, she deteriorated and was found to have lung metastases. Primary cardiac sarcomas, due to their rarity and poor prognosis, are often diagnosed at an advanced stage of the disease and lack sufficient data to establish a standard course of treatment. The cornerstone of therapy is surgical resection. However, novel therapeutic approaches must be developed. Primary cardiac tumors should be suspected in adult patients with progressive dyspnea, and a biopsy should also be done to determine the histopathological pattern of the mass and estimate the overall prognosis and outcomes.

Primary Cardiac Tumours Managed at a Large Tertiary Centre

Primary Cardiac Tumours Managed at a Large Tertiary Centre

Primary angiosarcoma under the guise of constrictive pericarditis: a case report

Primary cardiac tumors are histologically heterogeneous and grow from the heart tissues. Their diagnosis is difficult due to the absence of pathognomonic signs, as well as the possibility of a long asymptomatic course. The authors present a case of late diagnosis of heart tumor in a young man, occurring under the guise of constrictive pericarditis with rapidly progressive heart failure, which ended in death 3 months after the first clinical symptoms. According to the autopsy, the histological picture and immunophenotype of the tumor corresponded to angiosarcoma.

Diagnosis and treatment of cardiac angiosarcoma

Primary cardiac tumors are very rare in the population. About 20% of it are malignant. The most common type of cardiac malignant tumor is angiosarcoma. This tumor has the ability to infiltrate surrounding tissues and form distant metastases. Angiosarcoma is most often located in the right atrium and it mostly attacks men. This neoplasm is difficult to diagnose, because it has no specific symptoms. Patients with this tumor may complain of a chest pain, cardiac arrhythmias, suffocation and distal oedema. The rarity of occurrence and the lack of specyfic symptoms contribute to late diagnosis of the disease and worse prognosis of patients. Angiosarcoma can also be misdiagnosed as pneumonia or more common cardiovascular disease for example coronary artery disease. In advanced stage, this tumor can even lead to myocardial rupture. Various imaging methods are used in the diagnostics of this neoplasm for example: CT, MRI, PET-CT or Echocardiography. Histopathological examinations are less frequently used due to the risk of serious complications like hemothorax. Due to the very uncommon occurrence of the angiosarcoma, guidelines describing treatment of patients with this neoplasm, have not been developed yet. Currently, surgical removal of the tumor (if it is possible), chemiotherapy and radiotherapy are used. Reaserches on the genetic mutations which cause angiosarcoma and the development of appropriate drugs may give patients a chance for more effective treatment in the future

Intracardiac Metastasis of SCC Of Lung: Rare Unusual Incidental Finding on Autopsy Case Report

INTRODUCTION: Metastasic tumors of the heart are extremely rare and these cases are mostly diagnosed at autopsy. Although being rare, these tumors are 30 - 40 times more common than the primary cardiac tumors. The incidence of metastatic tumor of the heart is 1.5 to 20.6% which is gradually increasing in number. In the majority of carcinomas metastatic to the heart,the primary tumor is in the thoracic cavity or contiguous area, and the tumor reaches the heart by metastasizing to the mediastinal lymph nodes and from there it extends in a retrograde fashion to the cardiac lymph vessels. METHODOLOGY: Autopsy was conducted on a 40-year-old male patient who had died of a chronic unknown illness and his organs which included heart, lungs, liver, spleen and kidneys ere received in our department. OBSERVATION AND RESULTS: Microsections from lung and various portions of the heart showed a well diff erentiated SCCs with keratinization and sporadic keratin pearls. CONCLUSION: Metastasis to the heart develops very rarely as the initial presentation of a malignancy. Most of the patients have widespread metastasis and cardiac involvement is usually diagnosed at autopsy. Although cardiac metastasis from heart is rare, the possibility of the primary should be considered in the absence of a previous history, symptoms or even a discrete lesion.

Clinical trends of children with primary cardiac tumors: 15-year experience from a single center.

e22008 Background: Clinical presentation of pediatric primary cardiac tumors is exceptionally rare throughout the world. The purpose of our study is to evaluate clinical intervention by following up on children with primary cardiac tumors. Methods: Our study retrospectively enrolled 97 children diagnosed with primary cardiac tumors from Jan 2005 to Dec 2020 in a single center, Children’s Hospital of Nanjing Medical University by confirming with clinicoradiological and non-invasive diagnostic procedures. Primary outcome was to measure clinical trends and interventions while secondary was to analyze survival of 26 (26.80%) operative and 71 (73.19%) non-operative patients. Surgical intervention patients were followed for 6 Mo in the first postoperative year, then every year. Meanwhile, non-serious, asymptomatic, and no hemodynamic changes patients were treated non-operatively and followed up every year regularly. Results: In primary outcome, median age of children was 4.72±4.10 years (1d~16y), and median follow-up was 5.60±4.97 years (1m~15y). Most enrolled children were younger than 1 year old at the time of diagnosis. Primary cardiac tumors patients consisting of 60 rhabdomyoma (61.85%), 11 myxomas (11.34%), 7 fibromas (7.21%), 3 lymphangiomas (3.09%), 2 lipomas (2.06%), 9 unclassified tumors (9.27%) and 5 malignant tumors (5.15%) were retrospectively analyzed. Major 75 patients (77.31%) were diagnosed during various treatments of clinical symptoms, whereas 18 (18.55%) were diagnosed with routine investigation and 14 (14.43%) were diagnosed by prenatal fetal echocardiography. Herein, 26 children with critical symptoms and serious hemodynamic alteration had undergone surgical intervention in our hospital. Among them, 6 (23.07%) patients had postoperative complications; 2 (7.7%) patients were unable to survive due to post-operative arrhythmia and low cardiac output syndrome; While 2 (7.7%) patients with myxomas had a reoccurrence, and 2 (7.7%) patients had been diagnosed with another rhabdomyoma after intervention, others recovered well. Meanwhile, 71 children had non-surgical management, where 8 (11.26%) were lost to follow-up; 3 (4.22%) patients with malignant tumors were unable to survive after discharge due to unknown causes, and other patients had no serious symptoms or growth of tumors during follow-up. In secondary outcome, survival between operative and non-operative patients had no significant difference ( P &gt; 0.05). Conclusions: Clinical intervention of pediatric primary cardiac tumors may be performed one-on-one by accounting presentations, symptoms, and emergence even though majority of primary cardiac tumors in children are benign in nature, and postoperative reoccurrence is feasible, particularly for rhabdomyoma. Meanwhile, the primary goal of surgical intervention is to reinstate normal hemodynamics, and preserve significant structures and cardiac tissues.

Misdiagnosis of a Right Atrial Cardiac Haemangioma: A Case Report

Cardiac haemangiomas (CH) constitute 2.8% of primary cardiac tumours [ [1] Li W, Teng P, Xu H, Ma L, Ni Y. Cardiac Haemangioma; A comprehensive analysis of 200 cases. Ann Thorac Surg. 2015;99:2246–52. Google Scholar ]. Right atrial (RA) CHs are very rare. Despite being benign lesions, they may result in life-threatening complications including stroke and sudden death [ [1] Li W, Teng P, Xu H, Ma L, Ni Y. Cardiac Haemangioma; A comprehensive analysis of 200 cases. Ann Thorac Surg. 2015;99:2246–52. Google Scholar ]. Although advances in imaging have provided insight into this condition, preoperative misdiagnosis is common. Diagnosis and management of CH is limited by the scarcity of evidence. Surgical excision remains the standard of care [ [1] Li W, Teng P, Xu H, Ma L, Ni Y. Cardiac Haemangioma; A comprehensive analysis of 200 cases. Ann Thorac Surg. 2015;99:2246–52. Google Scholar ].

A new approach to the interpretation of B-type natriuretic peptide concentration in children with congenital heart disease

Abstract Objectives BNP is the unique cardiac marker that reflects not as much as the degree of heart muscle damage, but mostly the severity of hemodynamic disorder, which is important in congenital heart disease. The only disadvantage of this marker is the barely studied reference values for children. It is known that the younger the child is, the higher the BNP value will be. By shifting from interpreting the absolute values towards the application of zlog-transformed data in clinical practice, we can overcome the above problems. Methods We performed an age-adjusted zlog transformation of BNP concentration. The age dependence was accounted for by a piecewise linear interpolation of the logarithms of BNP concentration among healthy children in different age groups from the logarithms of age. Results The concentration of BNP was measured in 351 patients (under 1 year old) with various heart diseases. The median age at the time of testing was 52 days [10; 166]; the median weight was 4.1 kg [3.2; 6.2]. The conditions we investigated included almost all known congenital heart diseases, as well as primary cardiac tumors. After the zlog transformation, we eliminated age-dependence, which was proved by comparing BNP concentrations in two groups of patients with univentricular and biventricular hemodynamics. Conclusions BNP in patients with congenital heart disease reflects the severity of hemodynamic disorders, and zlogBNP is an objective, age-independent and clear mechanism that can be used to interpret this cardiac marker.

COVID-19 mortality in a patient with left atrial myxoma

Individuals with comorbid conditions, particularly those with cardiovascular diseases, are at a higher risk of mortality due to COVID-19. Among primary cardiac tumours, atrial myxoma is the most commonly reported, accounting for 85% of all cases. Most tumours occur in the left atrium with an associated embolic phenomenon in 40to 50% of patients. In this case report, we describe a 35-year-old woman of West African descent who had a left atrial myxoma and was waiting for surgery. She presented with signs and symptoms of biventricular heart failure, and her severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) test results were negative. She deteriorated and died while being optimized medically before cardiac surgery. Postmortem confirmed a left atrial myxoma, myocardial infarction, bacterial endocarditis, congestive cardiac failure, COVID-19 pneumonia, as well as a positive real-time Reverse Transcription Polymerase Chain Reaction (rRT-PCR) SARS-CoV-2 test on lung swabs. Though uncommon, cardiac myxomas may result in mortality if not managed promptly. COVID-19 may complicate the management of patients with atrial myxoma with mortality.

P74 NIGHT SWEATS AND AN UNUSUAL RIGHT ATRIAL MASS

Abstract Background Primary cardiac tumors are infrequently, usually benign, and diagnosed incidentally. Among primary cardiac tumors, cardiac hemangiomas are exceptionally rare with only a 1–2% incidence. In most cases, histological diagnosis is made after surgical removal or at autopsy. Case presentation: A 36–year–old male patient is suffering from several months of bothering night sweats. He has no previous medical history and is not taking medication. After performing various exams, including an electrocardiogram and chest rx–ray that were unremarkable, the patient performed a chest TC scan that shows a large mass at right atrial level (Figure 1). Surprisingly, at transthoracic echocardiography the mass was not visible and moreover the exam was normal. To better visualize the mass, a transesophageal echocardiography was performed, showing a inhomogeneous, round–shape, intracavitary mass of 70x65 mm in size. The mass appear to be fixed, expansive, with no embolic–features and seem to be initially–obstructing the upper–cava blood flow (Figure 2), even the patient was hemodynamically stable. A diagnosis of lymphoma was initially suspected and an Total–body PET–TC was performed, showing that the heart mass was metabolically inactive and an intestinal metabolic active mass. Intestine mass biopsy, made by colonscopy, showed a non–malignous villous adenoma. Before undergoing heart surgery, to understand the nature of the mass, we performed a intracavitary ecocardiography (ICE)–guided percutaneous biopsy. Histologically, the heart tumor was composed of cavernous channels embedded in a loose fibrous stroma, compatible with Cavernous hemangioma. At heart surgery, the mass was localized inside the right atrium, at upper–front level, adhered to a small part of atrial myocardium, and was removed without complications. The right atrium was rebuilt with artificial patch. (Figure 3). Conclusions Cavernous cardiac Hemangioma is a rare tumor that may have an atypical clinical presentation. To our knowledge, this is the first case of cardiac hemangioma diagnosed by ICE–guided endomyocardial biopsy.

P270 THE IMPORTANCE OF INTEGRATED IMAGING IN THE DIFFICULT DIFFERENTIAL DIAGNOSIS OF A LEFT INTRACARDIAC MASS IN YOUNG ADULT

Abstract The patient is a 34–year–old man who comes to the emergency room with mild hemoptysis, asthenia, low–grade fever, night sweats and weight loss. Cardiac auscultation reveals a diastolic murmur at the cardiac apex. Transthoracic and transesophageal echocardiograms are performed and show an isoechoic thickening encompassing the posterior leaflet, cranially involving the atrial septum and the lateral wall up to the inlet of the left atrial appendage and inferiorly involveing the subvalvular apparatus with diffuse thickening and chordal fusion. Functionally it determines a moderate mitral stenosis with an average gradient of 10 mmHg and a valve area of ​​1.1 cm2 and a mild–moderate regurgitation with an anteriorly directed eccentric jet. At this point we needed to make the differential diagnosis considering as possible etiologies infective endocardic vegetation, thrombosis, primary and secondary neoplasms, aseptic vegetation and inflammatory or rheumatic valvulitis. We therefore begin our diagnostic work–up by performing a scintigraphy with labeled leukocytes, which does not show the presence of hyperuptake areas referable to infective endocarditis, and a PET scan which shows, in addition to the increased uptake at the cardiac level, an area of ​​greater concentration of the metabolic tracer in the gastric area (between the body and the fundus). Given the confirmation of metabolically active tissue, the patient performed cardiac magnetic resonance with contrast medium which confirmed the presence of pathological tissue with inhomogeneous late post–contrast enhancement. After collegial discussion, due to the high risk of biopsy of the mitral lesion, which was poorly separable from the cardiac structures, given the other localization of the disease at the gastric level, it was decided to perform an EGDS with relief of an ulcerated lesion on the gastric fundus which is subjected to biopsy and histological typing. We then arrive at the diagnosis of “undifferentiated pleomorphic sarcoma”. Sarcoma is the most frequent histological type of primary malignant cardiac tumors. The clinic is characterized by constitutional symptoms, obstruction of the valve inflow or outflow tract, thromboembolism, and arrhythmias. It has a poor prognosis: 6–12 months from diagnosis. In conclusion, we reiterate the importance of integrated imaging to arrive at the final diagnosis, each method with a specific objective.