Primary Cardiac Tumors Research Articles

Incidence and survival rates of patients with primary malignant cardiac tumors: A SEER based study.

e24007 Background: Primary cardiac tumors (PCTs) are rare growths, comprising less than 1% of all cardiac tumors, with cardiac metastases occurring 30-times more frequently. The incidence of primary malignant cardiac tumors (mPCTs) has been observed to be approximately 10% of all PCTs. The majority of available data on PCTs have been obtained from post-mortem analyses. The advent of echocardiography and specialized cardiac radiography has improved characterization of these tumors. Despite technological advancements in cardiac imaging, there are limited studies on the incidence and survival rates of patients with mPCTs. Methods: The Surveillance Epidemiology and End Results (SEER) database was used to identify and characterize patients diagnosed with mPCTs. Data were collected from 17 registries ranging from the years 2000-2021 for patients with one primary tumor originating in the heart. Further characterization of patients including age, race, tumor histopathology codes, and survival time following diagnosis were included in analysis. Kaplan-Meyer curves and logistic regression analyses were used to identify potential differences in survival by subgroups. Results: Analysis of 445 patients, 200 female and 225 male, showed an overall average yearly incidence rate of 20 cases. Overall, there was no statistically significant difference in incidence rates across these years (p = 0.46). The most commonly diagnosed mPCT was hemangiosarcoma (41.65% of cases) followed by sarcoma, not otherwise specified (7.42% of cases), pleomorphic cell sarcoma (6.97% of cases), fibrosarcoma (5.16% of cases), and leiomyosarcoma (4.94% of cases). Of these patients, 21.1% presented with local disease, 24.7% with regional metastases and 31.0% with distant metastases. The average length of survival of those with death attributable to any mPCT (N=390) was 20 months. The median overall survival (OS) for this population was 11 months. There was no statistically significant difference in OS rates between the top five most common mPCT subtypes (p = 0.41). There was no significant difference in OS based on sex (p = 0.77) or race (p = 0.33). There was no statistically significant difference in 6-month, or 1-year, OS rates (62% and 48%; p = 0.25, p = 0.09, respectively) across time. Treatment with chemotherapy, radiation, and both chemoradiation did not have a statistically significant impact on OS rates (p > 0.4 for all treatment modalities). Conclusions: The incidence and OS of patients with mPCTs has remained consistent throughout the years 2000-2021 despite advancements in cardiac imaging. 6-month and 1-year survival rates of patients diagnosed with mPCTs have remained unchanged in this population. Further studies are indicated to determine the factors contributing to a lack of improvement in diagnosis and survival of this population.

THE DIAGNOSTIC CHALLENGE OF A RARE CASE OF CARDIAC MYXOMA: THE CRUCIAL ROLE OF ECHOCARDIOGRAPHY

THE DIAGNOSTIC CHALLENGE OF A RARE CASE OF CARDIAC MYXOMA: THE CRUCIAL ROLE OF ECHOCARDIOGRAPHY

Prevalence, Diagnosis, and Treatment of Cardiac Tumors: A Narrative Review.

Cardiac tumors, though rare, present significant diagnostic and therapeutic challenges due to their heterogeneous nature and anatomical complexity. This narrative review synthesizes current evidence on prevalence, diagnostic modalities, and management strategies for primary and metastatic cardiac tumors. Echocardiography, cardiac MRI, and CT remain cornerstone imaging tools for differentiating tumors from non-neoplastic masses, while advances in PET/CT and tissue characterization techniques refine staging and treatment planning. Surgical resection with clear margins (R0) is critical for resectable tumors, particularly benign myxomas, though malignant tumors like sarcomas require multimodal approaches combining surgery, radiotherapy, and systemic therapies. Emerging strategies such as heart autotransplantation and staged resections offer promise for complex cases, while oligometastatic disease management highlights the role of stereotactic radiotherapy and immunotherapy. Key challenges include standardizing resection margins, optimizing neoadjuvant therapies, and addressing high recurrence rates in malignancies. Future directions emphasize integrating AI-driven imaging analysis, molecular biomarkers, and genomic profiling to personalize therapies, alongside global registries to enhance data on rare tumors. Equitable access to advanced diagnostics and multidisciplinary collaboration are essential to improve outcomes. This review underscores the need for standardized guidelines, technological innovation, and patient-centered research to address gaps in cardiac oncology.

Brain Metastases from Primary Cardiac Tumors: A Systematic Review of Diagnosis, Treatment, and Prognosis.

Background: Primary cardiac tumors (PCTs) are rare entities, with only a minority being malignant and capable of distant dissemination. Among the rarest and most challenging metastatic events are brain metastases originating from cardiac tumors. Due to the heart's direct access to systemic circulation, even benign tumors such as atrial myxomas may cause cerebral embolic phenomena. Understanding the distinct biological behavior, diagnostic pathways, therapeutic strategies, and prognostic implications of these cases remains limited by the scarcity of the available literature. Methods: A systematic review was conducted following PRISMA guidelines. PubMed, Scopus, Embase, and other major databases were systematically searched using specific MeSH terms and keywords related to cardiac tumors and brain metastases. After applying strict inclusion and exclusion criteria, nineteen studies were included, comprising sixteen single-patient case reports and three multi-patient series. Extracted data included tumor histology, cardiac and brain imaging findings, neurological presentation, treatment approaches, and patient outcomes. Results: A total of 320 patients were analyzed. Atrial myxomas represented the predominant benign tumors causing embolic cerebral events, while angiosarcomas and other cardiac sarcomas were responsible for true hematogenous brain metastases. Brain involvement was frequently hemorrhagic and manifested with seizures, focal deficits, or signs of intracranial hypertension. Cardiac echocardiography and cardiac magnetic resonance imaging (CMR) were essential for tumor detection, while brain MRI, including SWI and DWI sequences, and CT scanning were critical for cerebral lesion characterization. Treatment strategies varied according to tumor type and included surgery, radiotherapy, and systemic therapies. Malignant cardiac tumors correlated with a poor prognosis, with median survival post-CNS involvement ranging from 12 to 14 months. Conclusions: Brain metastases from PCTs, though rare, represent a distinct and serious clinical phenomenon. Benign tumors like myxomas mainly cause embolic cerebral events, whereas malignant tumors, particularly sarcomas, lead to true metastatic brain lesions. Recognizing this biological distinction is crucial for diagnosis, prognostication, and therapeutic planning. An integrated multidisciplinary approach combining advanced cardiac and neuroimaging techniques is vital for early detection and appropriate management. Despite multimodal treatment, survival remains limited, underscoring the urgent need for novel targeted therapies and improved surveillance strategies.

Dynamic evolution of fetal cardiac rhabdomyomas: prenatal diagnosis and postnatal echocardiographic insights.

Cardiac rhabdomyomas require early prenatal recognition and genetic counseling to manage potential cardiac and extracardiac complications. A multidisciplinary follow-up approach enhances postnatal outcomes and informs the management of future pregnancies. • Fetal cardiac rhabdomyomas are the most common primary cardiac tumors in utero and are often associated with tuberous sclerosis complex. • This case series highlights the diverse postnatal tumor behaviors-including growth, stability, and regression-emphasizing the need for individualized follow-up.

Cystic tumor of the atrioventricular node: a case report

Primary cardiac tumors are rare anomalies, with an incidence ranging from 0.0017% to 0.03% in autopsies. Among them, atrioventricular (AV) node cystic tumors present as benign congenital anomalies located in the trigone of the atrioventricular node. This type of tumor accounts for approximately 2.7% of all primary cardiac tumors and has the potential to cause severe arrhythmias or sudden death. This article reviews AV node cystic tumors and discusses its etiology, symptoms, diagnosis, and treatment. Although its exact origin is still uncertain, it is considered a significant congenital anomaly because of its association with sudden cardiac death, which constitutes about 50% of cardiovascular mortality. The absence of visible macroscopic signs underlines the importance of maintaining a high index of suspicion in patients presenting with AV node localized heart block or sudden death, particularly in children, young adults, and women. Early detection and accurate diagnosis are critical, requiring a thorough evaluation of the cardiac conduction system and detailed tissue analysis. Careful investigation and prompt intervention can significantly improve clinical outcomes and reduce the risk of sudden cardiac death.

Cardiac Hemangiomas: A Five-Year Systematic Review of Diagnosis, Treatment, and Outcomes.

Background/Objectives: Cardiac hemangiomas are rare benign vascular tumors, accounting for less than 2% of primary cardiac tumors. Despite their rarity, they can cause significant clinical effects depending on their size and location. This systematic review aims to provide an updated analysis of recent cases, focusing on epidemiology, histopathological subtypes, clinical presentation, diagnostic approaches, and treatment outcomes. Methods: A systematic search of the PubMed database identified case reports and series published between 2019 and 2025. After applying inclusion and exclusion criteria, 55 eligible cases were selected for analysis. Data were extracted on patient demographics, tumor characteristics, imaging methods, treatment strategies, and follow-up outcomes. Results: Cavernous hemangiomas were the most commonly reported subtype. Patient ages ranged from infancy to over 85 years, with a slight predominance in females. Presentations varied from asymptomatic incidental findings to symptoms such as dyspnea and chest pain. Echocardiography was the primary diagnostic tool in over 80% of cases. Surgical resection was performed in 87.3% of patients, yielding favorable outcomes and low recurrence. However, follow-up duration was inconsistent, and long-term outcomes were underreported, underscoring the lack of standardized surveillance protocols. This variability limits clinicians' ability to assess recurrence risk and long-term prognosis, highlighting the need for evidence-based guidelines to inform structured follow-up and improve patient care. Conclusions: The increasing number of published cases likely reflects improved diagnostic imaging and greater clinical awareness. While surgery remains the preferred treatment, the variability in follow-up and diagnostic reporting highlights the need for standardized protocols. Further studies are warranted to clarify the natural history, refine classification systems, and establish evidence-based guidelines for the management of this rare cardiac tumor.

Primary Ewing Sarcoma in the Right Ventricle in an Adult Patient: A Case Report

Ewing sarcoma, a highly aggressive round-cell neoplasm of unknown origin, rarely occurs as a primary cardiac tumor. In this intriguing case, we describe an instance of primary Ewing sarcoma in an adult patient’s right ventricle (RV). The patient, a 27-year-old man, presented with symptoms including epigastric pain, fever, tachycardia, nausea, vomiting, shortness of breath, and a dry cough. Imaging studies, including a computed tomography scan and echocardiogram, revealed an anterior mediastinal mass arising from the free wall of the RV and extending into the pericardium. The mass exerted pressure on the RV outflow tract and the pulmonary artery. Surgical intervention was deemed necessary based on the clinical presentation and paraclinical findings. The mass was meticulously dissected from the cardiac tissue, and the RV outflow tract was resected, along with the pulmonary valve and the main pulmonary artery. These structures were replaced with a composite Dacron graft, anastomosed to the bifurcation of the main pulmonary artery. Additionally, the mass near the superior vena cava and the pulmonary vein region was carefully excised. The patient was successfully weaned off the cardiopulmonary bypass pump in stable condition and transferred to the ICU. Histopathological analysis confirmed the diagnosis of small round cell sarcoma, specifically Ewing sarcoma. Following recovery, the patient was discharged and scheduled for regular follow-up appointments. Following discharge, the patient was referred to a radiotherapy center and underwent radiotherapy. During a follow-up examination 1 year later, no signs of disease recurrence or progression were observed, offering a promising outlook. This case underscores the importance of timely referral and treatment for cardiac Ewing sarcoma, which may significantly improve prognosis.

Clinical Features of Antiphospholipid Syndrome with Intracardiac Mass.

Antiphospholipid syndrome (APS), a disorder characterized by the presence of antiphospholipid antibodies, is commonly associated with thrombotic events and pregnancy complications. Although cardiac involvement of APS is very common, intracardiac thrombus is rare and easily misdiagnosed. In order to reduce missed diagnosis and misdiagnosis, we investigated the clinical features of APS with intracardiac mass by summarizing 50 cases (1 newly presented case and 49 additional cases collected from PubMed from 1985 to the present). There were 10 males and 40 females, with ages ranging from 8 to 75 years (median age 35.5). Intracardiac masses were distributed in four cardiac chambers. Mass size ranged from a diameter of 0.5 to 7.1 cm. Clinical manifestations were heterogeneous, including dyspnea, fever, hemiparesis, limb ischemia, and other nonspecific symptoms. In 41 cases with available pathology results, 33 cases were confirmed as thrombus, 2 cases as myxoma, 3 cases as non-bacterial endocarditis, 2 cases as fibrous tissue, and 1 case as inflammatory necrosis. Among 41 cases, 18 cases were suspected of primary cardiac tumors preoperatively, while pathological examination revealed none was tumor. APS patients with intracardiac masses are extremely rare, mostly seen in young or middle-aged people, and they present with a variety of clinical manifestations. Most masses disappear following medical treatment. APS can be accompanied by cardiac myxomas. APS should be promptly investigated in young patients presenting with thrombotic events without any underlying risk factors.

A Study of Clinical and Pathological Spectrum of Primary Cardiac Tumors: A Clinical Study at a Dedicated Cardiac Center of Western Part of India

A Study of Clinical and Pathological Spectrum of Primary Cardiac Tumors: A Clinical Study at a Dedicated Cardiac Center of Western Part of India

A Common Primary Cardiac Tumor With an Uncommon Presentation

A Common Primary Cardiac Tumor With an Uncommon Presentation

Clinical characteristics and surgical outcomes following cardiac myxoma resection.

Cardiac myxomas are the most common primary cardiac neoplasm (30-50%) with clinical incident of 0.5/ million population. Tranthoracic echocardiography remains the investigation of choice. Surgical excision is curative. The present study aims to analyze demographic and clinical characteristics as well as surgical outcomes in terms of mortality and recurrence of cardiac myxoma. Thirty patients of cardiac myxoma who met the inclusion criteria during study period study period, January-2018 to April-2024 were included. Data was analyzed for demographic characteristics, echocardiographic findings of myxoma and associated valve lesion, associated valve surgery and survival outcome. Of all subjects, 83.33% presented with dyspnea. Majority of myxoma, 76.67% were attached to interatrial septum. Overall survival at 1- and 3- year was 91.23%. Recurrence free survival at 1-, 3- years and end of this study were 100%, 84.71% and 84.71% respectively. Myxomas with valvular incompetence are rare entity and there is paucity of data and evidences recommending concomitant valve intervention in such cases. There were no immediate peri-operative deaths, however, in contrast to other studies; surgical site infection was the most common post operative complication. Overall survival at 1- and 3- year was 91.23%. Recurrence free survival at 1-, 3- years and end of this study were 100%, 84.71% and 84.71% respectively. Recurrence occurred in first- and third-year following surgery. Study highlights decent outcomes following cardiac myxoma resection. Case specific concomitant valve intervention spiral the success of surgery.

Safety and Efficacy of Unresectable Malignant Cardiac Tumors Treated with Concurrent Chemoradiation Therapy Using a 1.5T MR-Linac (GASTO-1078).

Safety and Efficacy of Unresectable Malignant Cardiac Tumors Treated with Concurrent Chemoradiation Therapy Using a 1.5T MR-Linac (GASTO-1078).

The impact of thyroid function on surgical prognosis in patients undergoing cardiac and major vascular surgeries

ObjectiveThis study aims to elucidate the relationship between thyroid function and surgical prognosis in patients undergoing cardiac and major vascular surgeries.MethodsA retrospective cohort study was conducted on patients undergoing cardiac or major vascular surgeries. Preoperative thyroid function tests, including TSH, free T3, and free T4 levels, as well as postoperative thyroid function tests, were assessed. Key postoperative outcomes, such as total hospital stay, postoperative hospital stay, ICU stay, and duration of mechanical ventilation, were recorded and analyzed. The analytical approach included Pearson correlation, multivariable logistic regression models, and restricted cubic splines.ResultsThis study analyzed a cohort of 472 patients who underwent various cardiovascular surgeries, including coronary artery bypass grafting (173 patients), aortic surgery (131 patients), valve surgery (125 patients), and primary cardiac neoplasms resection (43 patients). Significant changes in thyroid hormone levels were observed preoperatively and postoperatively, with TSH, FT3, and FT4 levels showing a significant decrease from preoperative values (p < 0.001). Multivariate logistic regression analysis further revealed that ΔTSH is significantly associated with total hospital stay, postoperative hospital stay, and duration of mechanical ventilation; postoperative FT3 levels were significantly inversely related to total hospital stay, ICU stay, and ventilator requirements. Additionally, although no significant nonlinear relationships were found (all p > 0.05).ConclusionsThyroid dysfunction may impact postoperative outcomes in cardiac and major vascular surgery patients.

Multiple papillary fibroelastoma presenting with mitral, tricuspid, and pulmonary valve involvement and surgical treatment: case report.

Approximately 90% of primary paediatric cardiac tumours are benign lesions. Depending on their location and size, benign cardiac tumours may cause inflow and outflow obstructions, cyanosis, valvular insufficiencies, myocardial ischaemia, associated dysfunction, systemic embolisation, arrhythmias, and even sudden death. Decision-making and timing for surgery can be challenging in children. Herein, we present an asymptomatic 11-year-old girl with papillary fibroelastoma in the mitral, tricuspid, and pulmonary valves, discussing the decision-making process and successful surgical management.

Prenatal Detection and Monitoring of an Intracardiac Teratoma

Case Report: This case report outlines the prenatal diagnosis and monitoring of an intracardiac teratoma initially detected during imaging studies in the second trimester. Our patient, a 28-year-old G2P1001 with an unremarkable medical, obstetric, and family history, underwent a routine anatomy ultrasound revealing abnormal cardiac findings including pericardial effusion, a possible double outlet right ventricle, and an enlarged aorta. Subsequent investigation by fetal echocardiogram at 23 weeks and 3 days confirmed the presence of an intracardiac teratoma. The large, heterogenous right atrial teratoma was already inducing structural and hemodynamic alterations throughout the fetal heart at the time of diagnosis. Our multidisciplinary team, in collaboration with Pediatric Cardiologists, determined a surveillance protocol for the patient, comprising weekly fetal echocardiograms, middle cerebral artery (MCA) and ductus venosus (DV) dopplers, and biophysical profile (BPP) assessments. This monitoring aimed to detect signs of decreased fetal cardiac output, fetal hydrops, fetal demise, or the development of maternal mirror syndrome. Our delivery plan was for a primary cesarean section at a specialized pediatric cardiac care facility, with consideration for an ex-utero intrapartum treatment (EXIT) procedure or pericardial drain placement, followed by postnatal cardiac surgery as indicated. Our patient is currently 36 weeks and outcome is pending. While primary cardiac tumors are rare, advancements in imaging technology and standardized screening practices may facilitate earlier detection, as demonstrated in this case, potentially leading to improved outcomes through appropriate management strategies. This case speaks to the success of our surveillance protocol and delivery planning.

Calcified Amorphous Tumor of the Mitral Valve Causing Stroke: A Rare Case of Heart Tumor

Abstract Calcified amorphous tumor (CAT), first described in 1997, is a rare primary cardiac tumor with a lifetime incidence of 0.0017%–0.02%, accounting for approximately 2.5% of all primary cardiac tumors. CAT can be discovered incidentally or following neurological events. Here, we report a poststroke patient diagnosed with filamentous formation on the mitral valve. After the surgery, the biopsy identified it as a CAT. There was a debate among colleagues regarding the indication for surgery due to the small mass. The prevalence of valvular strands is from 5.5% in the general population to 40% in patients with unexplained stroke. The incidence of systemic embolism, including cerebral, retinal, and transient ischemic attacks, is reported at 53%. Considering the significant risk of cerebrovascular accidents associated with calcified amorphous or similar masses, especially in patients with a history of stroke, surgery remains the treatment of choice.

CARDIAC TUMORS: UPDATED CLASSIFICATIONS AND MAIN CLINICO-PATHOLOGIC FINDINGS.

This review article - which is devoted to a wide range of physicians, e.g., pathologists, clinicians, radiologists, and general practitioners - is an up-to-date clinico-pathological description of cardiac tumours. Although rare, cardiac tumours are key components in oncology practice since both their early diagnosis and appropriate management denote urgent needs. Primary cardiac tumours (PCTs) are categorized in recent WHO classifications as well as in other scientific contributions. Their incidence is around 0.02% while their prevalence is between 0.001% and 0.03%. Among PCTs, benign lesions account for 85% of cases, while malignant neoplasms for 15%. Compared to PCTs, secondary cardiac tumours are 20-30-fold more common. The most frequent PCTs in adulthood are papillary fibroelastoma and cardiac myxoma, while, in childhood, cardiac rhabdomyoma. Heart metastases may occur through direct extension, or, indirectly, via bloodstream, lymphatics or intracavitary diffusion. Thoracic cancers (e.g. from pleura, lung, breast) are the most frequent cause of cardiac metastasis followed by oesophageal and haematological malignancies. Symptoms of PCTs (e.g. arrhythmias, dyspnoea, chest discomfort, syncope) are usually the result of both their regional involvement and location. Non-invasive imaging techniques (e.g. echocardiography, MRI, CT) and biopsy are key means in delineating tumour characteristics, size, and adjacent structure involvement. Most PCTs require surgery, which is recommended to prevent life-threatening complications. While many benign cardiac neoplasms may be completely resected, the treatment of choice for malignant PCTs and metastases is a combination of surgery, radio- and/or chemotherapy, as well as new alternative treatments, which may prolong survival in a small patient subset.

Accuracy of transthoracic echocardiography in diagnosis of cardiac myxoma: single center experience.

The differential diagnosis of cardiac myxomas (CM), the most common benign primary cardiac tumors, is broad and a thorough diagnostic workup is required to establish accurate diagnosis prior to surgical resection. Transthoracic echocardiography (TTE) is usually the first imaging modality used for diagnosis of suspected CM. In a single tertiary centre study, we sought to determine the accuracy, sensitivity, and specificity of TTE in the diagnosis of CM and to determine echocardiographic characteristics indicative of CM. We retrospectively analyzed clinical, echocardiographic, and pathohistological findings of 73 patients consecutively admitted for suspected CM. After diagnostic workup, 53 (73%) patients were treated surgically at our institution. Based on preoperative TTE, patients were divided into a CM group (n=45, 85%) and non-myxoma (NM) group. Of the 53 pathohistological specimens obtained during surgery, 39 (73%) were CM. The sensitivity and specificity of preoperative echocardiography were 97% and 50%, respectively. The overall accuracy was 85%. All NM tumors were found in an atypical location and 72% of CM were found in a typical position in the left atrium (p < 0.001). Tumors in NM group were significantly smaller than CM (24.3 ± 13.2 mm vs. 37.9 ± 18.3 mm, p = 0.017). Our study confirms very good accuracy of TTE in the diagnosis of CM. The most important echocardiographic characteristics to differentiate between CM and tumors of different etiology are tumor location and size. Smaller tumors presenting at an atypical location are less likely to be diagnosed as CM, and these require additional imaging modalities for accurate diagnosis.

Unraveling the mystery: Giant left atrial myxoma neovascularization through the right coronary artery

ABSTRACT Primary cardiac tumors are exceedingly rare, with an incidence ranging from 0.001% to 0.03% in autopsy studies. Among these, cardiac myxomas are the most common in adults, representing approximately half of all benign cardiac tumors. A retrospective study at the mayo clinic, involving 323 patients who underwent surgical resection of primary cardiac tumors between 1957 and 2006, revealed that 94% of these tumors were benign, with myxomas accounting for 50% of the cases. Myxomas predominantly occur in the atria, particularly in the left atrium. Although patients with myxomas may be asymptomatic, they can present with a triad of symptoms: embolism, intracardiac obstruction, and constitutional symptoms. The latter, which include low-grade fever, malaise, and decreased appetite, is attributed to the gradual release of inflammatory cytokines such as interleukin (IL)-1, IL-6, and tumor necrosis factor-alpha from the tumor. Understanding the vascularity of the tumor is crucial for surgeons, as knowing the blood supply and vascular territory can help minimize and control blood loss during surgery.