Cardiac benign metastatic leiomyoma (BML) is a rare cardiac tumor that is usually asymptomatic, frequently misdiagnosed and may result in serious complications, including embolization, heart failure and death. This review highlights the importance of considering cardiac BML in the differential diagnosis of cardiac masses, especially in women with a history of uterine leiomyomas. This review summarizes the current knowledge about cardiac BML, including its demographics, clinical presentation, etio-pathogenesis, diagnosis, and management. The authors discuss the challenges associated with diagnosing cardiac BML and emphasize the importance of a thorough history, physical examination, and imaging studies. They also review the different treatment options for cardiac BML, including surgical resection and role of medical and surgical castration. Early diagnosis and management of cardiac BML is crucial to prevent complications. This review provides valuable insights for clinicians who may encounter this rare condition. By raising awareness of cardiac BML and its management strategies, this review can improve patient care and outcomes.

Cardiac lymphangiomas represent one of the rarest types of cardiac tumors. We describe the case of a 28-year-old male with cystic lymphangioma and hypertrophic cardiomyopathy. There have been only a few cases reported and to the best of our knowledge this is the first documented case associating a cardiac lymphangioma with cardiomyopathy.

Burkitt's lymphoma (BL) is the most common subtype of non-Hodgkin lymphoma in children, typically presenting with extranodal masses in areas such as the abdomen, face, and neck. Cardiac involvement in BL is extremely rare, especially in pediatric patients, and has significant implications for diagnosis, management, and prognosis.

A 6-year-old male patient with Burkitt's lymphoma, presenting with an uncommon infiltration of the interatrial septum, is described. The patient initially presented with a painful right facial mass, bilateral periorbital edema, and a history of fever, weight loss, and lower limb pain. Imaging revealed extensive disease, including a right masticator muscle mass, mandibular infiltration, and multiple soft tissue and skeletal lesions, along with significant thickening of the interatrial septum. A diagnosis of Stage III Burkitt's lymphoma was confirmed through biopsy and immunohistochemical analysis. The patient was treated according to the ANHL1131 protocol, including Rituximab and multi-agent chemotherapy. Remarkably, the cardiac infiltration showed substantial regression on follow-up echocardiography after chemotherapy.

This case underscores the importance of a comprehensive cardiac evaluation in pediatric patients with Burkitt's lymphoma, particularly when presenting with atypical manifestations such as interatrial septum involvement. The findings demonstrate that systemic chemotherapy can effectively treat cardiac infiltration, even in complex cases with extensive disease. This report highlights the need for awareness of rare cardiac presentations in pediatric lymphoma and the critical role of advanced imaging modalities in diagnosis and management. Continuous monitoring and early implementation of cardioprotective strategies are essential to optimize outcomes in such cases.

Patients with acute pulmonary embolism (PE) have a wide spectrum of clinical presentations, from incidental findings to sudden cardiac death. Management and treatment recommendations in currently available guidelines vary according to patient risk and haemodynamic profile. A clot-in-transit (CiT) in the right heart chambers may be occasionally identified and is, therefore, an under-recognised but challenging condition, often preceding an abrupt clinical deterioration, and associated with increased mortality. Data on the detection of a CiT are sparse but consistent in attributing negative prognostic relevance, and therefore the presence of CiT should be systematically investigated and recorded in the setting of PE.In this review, the challenges related to the identification of a CiT are highlighted. Here, we propose an algorithm where the role of the Pulmonary Embolism Response Team (PERT) is reinforced. The PERT should convene once the CiT is suspected, to define the timeline for the diagnostic steps and subsequent management on a case-by-case basis. A patient with PE and CiT requires close bedside monitoring and rapid escalation therapy in case of clinical deterioration. Beyond anticoagulation alone, more aggressive strategies can be considered, including systemic thrombolysis, surgical pulmonary embolectomy and the currently emerging catheter-directed therapies. PROSPERO registration number: CRD42024493303.

Cardiac magnetic resonance (CMR) parametric mapping is underexplored in cardiac tumours. To evaluate the contribution of mapping sequences on the characterization of paediatric tumours.

All paediatric patients referred for cardiac tumours at Bambino Gesù Children's Hospital from June 2017 to November 2023, who underwent CMR with mapping sequences, were included. The diagnosis of tumour type was performed according to signal characteristics on different sequences. Mass parametric mapping for each subtype and interobserver variability was assessed. Sixteen patients were enrolled. The mean age at CMR was 7 ± 5 years. 'Traditional' mass type assessment diagnosed haemangioma (Group A) in three patients (19%), fibroma (Group B) in four patients (25%), rhabdomyoma (Group C) in six patients (37%), and lipoma (Group D) in three patients (19%). The analysis of variance analysis revealed significant differences in mass native T1 and mass extracellular volume (ECV) values among the four subgroups (P < 0.001 for both comparisons). The mean native T1 and ECV values were respectively 1465 ± 158 ms and 54 ± 4% for Group A, 860 ± 118 ms and 93 ± 4% for Group B, 1007 ± 57 ms and 23 ± 5% for Group C, and 215 ± 13 ms and 0 ± 0% for Group D.

Mass mapping analysis is feasible and reproducible in children. ECV values provide the most accurate differentiation. Mass ECV consistently resembles normal myocardium in rhabdomyoma, is extremely high (approaching 100%) in fibroma, equals to zero in lipoma, and matches blood pool ECV (1-Hct) in haemangioma.

Even if rarely detected, right atrial (RA) masses represent a diagnostic challenge due to their heterogeneous presentation. Para-physiological RA structures, such as a prominent Eustachian valve, Chiari's network, and lipomatous atrial hypertrophy, may easily be misinterpreted as pathological RA masses, including thrombi, myxomas, and vegetations. Each pathological mass should always be correlated with adequate clinical, anamnestic, and laboratory data. However, the differential diagnosis between pathological RA masses may be challenging due to common constitutional symptoms, as in the case of vegetations and myxoma, which present with fever and analogous complications such as systemic embolism. The implementation of transthoracic echocardiography (TTE) with pulsed wave (PW) tissue Doppler imaging (TDI) may improve the visualization and differentiation of intracardiac masses through different color coding of the pathological structure compared to surrounding tissue. More remarkably, PW-TDI can provide a detailed assessment of the specific pattern of motion of each intracardiac mass, with important clinical implications. Specifically, a TDI-derived pattern of incoherent motion is typical of right-sided thrombi, myxomas, and vegetations, whereas right-sided pseudomasses are generally associated with a TDI pattern of concordant motion synchronous with the cardiac cycle. An increased TDI-derived mass peak antegrade velocity may represent an innovative marker of the embolic potential of mobile right-sided pathological masses. During the last two decades, only a few authors have used TTE implemented with PW-TDI for the characterization of intra-cardiac masses' morphology and mobility. Herein, we report two clinical cases of totally different right-sided cardiac masses diagnosed using a multimodality imaging approach, including PW-TDI, followed at our institution. The prevalence and physiopathological characteristics of the most relevant RA masses and pseudomasses encountered in clinical practice are described in the present narrative review. In addition, we will discuss the principal clinical applications of PW-TDI and its potential value in improving the differential diagnosis of pathological and para-physiological right-sided cardiac masses.

Cardiac hemangioma is a rare, benign vascular primary tumor. Clinical presentation is either asymptomatic or with symptoms due to its location and spatial interaction with adjacent structures. The authors present a case of right cardiac hemangioma whose clinical diagnosis was triggered by symptoms of anasarca status, hepatic damage with ascites, pleural effusion and right heart failure. The 79 years-old patient has been treated with complete resection of the tumor by means of cardiopulmonary bypass, without complications. The mid-term outcome (12 months) was favorable. The aim of this study is to report a case of cardiac hemangioma with relevant dimensions, in a rare location (tricuspid valve) with acute onset and interesting aspects of clinical presentation.

Pericardial haemangiomas represent a very rare subset of benign cardiac tumour in an unusual location, posing a diagnostic and clinical challenge. Historically, the definitive diagnosis was achieved through surgical resection or at biopsy. In recent years, multi-parametric cardiac magnetic resonance imaging (MRI) has proven to offer a non-invasive, biopsy-like approach to tumour characterization.

In our case, multimodality imaging was used to characterize a pericardial mass as a haemangioma discovered coincidentally with a brain glioma. Diagnostic certainty was substantially improved through utilization of successive post-contrast bright-blood imaging at cardiac MRI, demonstrating a characteristic enhancement pattern of haemangiomas in direct comparison to the blood pool. Conservative management and mid-term follow-up showed an uneventful clinical course and partial regression of the presumed pericardial haemangioma.

In the presence of typical features and application of individually tailored protocols, multimodality imaging can characterize cardiac tumours and guide patient management so that more invasive measures may be avoided. In our case of a suspected pericardial haemangioma, a conservative strategy was adopted with clinically uneventful course over a 2-year period. Whether this strategy can be applied to other patients with this rare tumour remains unclear, but the case report provides important information about the natural history of this entity and tissue characterization by cardiac MRI.

Undifferentiated pleomorphic cardiac sarcomas are extremely rare, highly malignant mesenchymal cardiac neoplasms typically presenting in the sixth decade of life. Here, we have reported a rare case of undifferentiated pleomorphic cardiac sarcoma presenting with dyspnea in a young male. On further evaluations, a large, ill-defined, multilobulated mass was observed in left atrium (LA) with encasement of left circumflex artery, attachment to mitral valve annulus, and invasion through LA wall and pericardium.

Caseous mitral annulus calcification is a rare benign condition that can be misdiagnosed on echocardiography especially when it presents as a mass. This report highlights the contribution of cardiac MRI and computed tomography to the diagnosis through the case of a patient previously treated for breast cancer.

We report the case of a patient, previously treated for breast cancer, in whom echocardiography suggested the diagnosis of a cardiac tumor due the presence of a mass on the posterior mitral annulus. Cardiac magnetic resonance was inconclusive. Computed tomography confirmed the diagnosis of caseous mitral annulus calcification.

Cardiac angiosarcoma is a very rare and aggressive primary cardiac tumor associated with poor prognosis. Diagnosis is often delayed due to non-specific symptoms, with most cases involving metastases at the time of diagnosis. We describe a unique case of apparent tumor regression of cardiac angiosarcoma post percutaneous biopsy.

A young male was admitted with suspected pericarditis. Echocardiogram revealed a pericardial mass. Cardiovascular magnetic resonance (CMR) suggested primary cardiac malignancy. Percutaneous biopsy was inconclusive, with subsequent CMR demonstrating apparent tumor regression. Interval imaging revealed further tumor growth, and surgical biopsy revealed primary cardiac angiosarcoma (PCAS). Causes of tumor regression following percutaneous biopsy are discussed.

Cases of suspected primary cardiac malignancy require careful follow up with serial multimodality imaging. Percutaneous biopsy effects should be considered in cases of tumor regression, and serial imaging should be planned afterwards.

Cardiac myxomas are the most common benign tumors of the heart, with clinical manifestations varying significantly based on tumor size. Symptoms can range from asymptomatic and mild non-specific presentations to severe obstructive cardiac and systemic findings. This case report describes a 68-year-old female patient who presented with acute decompensated heart failure. Diagnostic evaluation revealed a left atrial myxoma causing significant mitral valve obstruction. The patient underwent emergency cardiac surgery for tumor removal, complicated by severe mitral and tricuspid valve regurgitation. Following valve replacement and repair, the patient required extracorporeal life support. Despite these complexities, she achieved significant recovery and was discharged in good condition. At follow-up, she remained asymptomatic with no signs of cardiac decompensation. This case highlights the importance of considering cardiac myxoma as a differential diagnosis in such cases to prevent potential complications.

Cardiac tumors, although rare, carry high morbidity and mortality rates. They are commonly first identified either at echocardiography or incidentally at thoracoabdominal CT performed for noncardiac indications. Multimodality imaging often helps to determine the cause of these masses. Cardiac tumors comprise a distinct category in the World Health Organization (WHO) classification of tumors. The updated 2021 WHO classification of tumors of the heart incorporates new entities and reclassifies others. In the new classification system, papillary fibroelastoma is recognized as the most common primary cardiac neoplasm. Pseudotumors including thrombi and anatomic variants (eg, crista terminalis, accessory papillary muscles, or coumadin ridge) are the most common intracardiac masses identified at imaging. Cardiac metastases are substantially more common than primary cardiac tumors. Although echocardiography is usually the first examination, cardiac MRI is the modality of choice for the identification and characterization of cardiac masses. Cardiac CT serves as an alternative in patients who cannot tolerate MRI. PET performed with CT or MRI enables metabolic characterization of malignant cardiac masses. Imaging individualized to a particular tumor type and location is crucial for treatment planning. Tumor terminology changes as our understanding of tumor biology and behavior evolves. Familiarity with the updated classification system is important as a guide to radiologic investigation and medical or surgical management. ©RSNA, 2024 Supplemental material is available for this article.

The crista terminalis is an anatomical structure localized on the posterolateral wall of the right atrium (RA). We performed a systematic review of the literature and meta-analysis concerning cases of unusual prominent crista terminalis mimicking RA mass. Moreover, we described the differential diagnosis of cardiac masses with the use of echocardiography, computed tomography, and cardiac magnetic resonance (CMR). We also emphasize the potential importance of this structure in electrophysiological procedures, including its role in exaggerated arrhythmias. Prominent crista terminalis may be a potential obstacle during invasive cardiac procedures or catheter ablation target. In analyzed cases, the crista terminalis was often erroneously interpreted as pathologic and at first confused with a thrombus or tumor during transthoracic echocardiography examination. The correct final diagnoses were mostly made with used transesophageal echocardiography or CMR. The most important imaging findings suggestive of prominent crista terminalis rather than tumor were a similar echogenicity/intensity with adjacent myocardium, the location on posterolateral wall of the RA, the phasic change in size, and no enhancement after contrast injection. We describe up to date and detailed imaging features for the differential diagnostics of selected intracardiac masses using various imaging techniques, including multimodality cardiac imaging. Familiarity with the anatomy and the imaging findings of the prominent crista terminalis will reduce misdiagnosis and avoid additional tests and unwarranted clinical interventions, while in patients considered for invasive cardiac procedures it might increase their efficacy and safety.

This article discusses the rarity of primary cardiac tumors, particularly angiosarcomas, accounting for 10% of primary cardiac neoplasms. The article highlights the challenge of early diagnosis due to nonspecific symptoms. The importance of sectional imaging, particularly computed tomography (CT) scans, in emergent situations is emphasized. Teaching point: Despite the rarity of primary cardiac tumors, radiologists should remain alert, especially when pericardial effusion is present.

Ventricular hemangiomas are rare benign tumors, pose diagnostic and therapeutic complexities. We report a case of a 52-year-old female with essential hypertension who developed a systolic ejection murmur during a hypertension clinic visit. The echocardiogram revealed a hyperechoic mass obstructing the right ventricular outflow tract, causing enlargement of the right atrium and ventricle, with a reduction in the right ventricular ejection fraction. Due to the risk of death, the patient underwent an emergency surgical resection along with tricuspid valve replacement. Postoperative recovery was uneventful, and subsequent cardiac magnetic resonance imaging showed an improvement in ejection fraction without residual tumor. This case highlights the diagnosis and therapeutic complexities of ventricular hemangiomas. With this report, we aim to provide a comprehensive review of ventricular hemangiomas and to enhance understanding of this condition for improved patient care.

Right-side heart mass can be found incidentally on routine transthoracic echocardiography (TTE). Accurate diagnosis of cardiac mass often requires more than one imaging method. We present a mid-age woman with non-Hodgkin lymphoma who was found to have multiple right atrial masses mimicking metastases on routine TTE, which were finally diagnosed as thrombi by multimodal cardiac imaging.

A 52-year-old woman was diagnosed with primary mediastinal diffuse large B cell lymphoma (DLBCL) almost six months prior. The TTE revealed multiple masses in the right atrium with normal cardiac function when she was being evaluated for the next chemotherapy. On arrival, she was hemodynamically stable and asymptomatic. Physical examination was no remarkable. Laboratory findings showed leukocytosis of 17,900 cells/mm3, hemoglobin of 7.5 mg/dL, and a normal D-dimer level. The suspicious diagnosis of right atrial metastasis was made by TEE. However, the diagnosis of right atrial thrombi was made by contrast CMR. Finally, the 18 F-FDG PET-CT demonstrated no metabolic activity in the right atrium, which further supported the diagnosis of thrombi. Eventually, the masses were removed by cardiopulmonary bypass thoracotomy because of a high risk of pulmonary embolism. Histopathology confirmed the diagnosis of thrombi.

This case highlights the importance of multimodality cardiac imaging in the appropriate diagnosis of a RA masses in patient of lymphoma. Diagnosis of RA masses can be made using multimodal cardiac imaging like TTE, TEE and CMR, even PET. Echocardiography is the most commonly used on multimodal imaging in cardiac thrombus. CMR has high specificity in differentiating a tumor from thrombus, while 18 F-FDG PET has good sensitivity to determine the nature of the masses.

This case report details a challenging instance of undifferentiated/unclassified cardiac sarcoma in a 28-year-old female, presenting with diverse symptoms like muscle weakness, shortness of breath, and hemoptysis. Diagnostic hurdles led to an initial misdiagnosis of granulomatosis with polyangiitis before discovering a sizable left atrial mass, ultimately diagnosed as high-grade undifferentiated/unclassified sarcoma. Despite initial surgical intervention, the patient's condition worsened, underscoring the complexities in managing such cases involving cardiac sarcomas. This case emphasizes the diagnostic complexities associated with primary cardiac sarcomas, particularly the challenges in achieving accurate diagnoses and formulating effective treatment strategies.

Multimodality imaging is currently suggested for the noninvasive diagnosis of cardiac masses. The identification of cardiac masses' malignant nature is essential to guide proper treatment. We aimed to develop a cardiac magnetic resonance (CMR)-derived model including mass localization, morphology, and tissue characterization to predict malignancy (with histology as gold standard), to compare its accuracy versus the diagnostic echocardiographic mass score, and to evaluate its prognostic ability.

Observational cohort study of 167 consecutive patients undergoing comprehensive echocardiogram and CMR within 1-month time interval for suspected cardiac mass. A definitive diagnosis was achieved by histological examination or, in the case of cardiac thrombi, by histology or radiological resolution after adequate anticoagulation treatment. Logistic regression was performed to assess CMR-derived independent predictors of malignancy, which were included in a predictive model to derive the CMR mass score. Kaplan-Meier curves and Cox regression were used to investigate the prognostic ability of predictors.

In CMR, mass morphological features (non-left localization, sessile, polylobate, inhomogeneity, infiltration, and pericardial effusion) and mass tissue characterization features (first-pass perfusion and heterogeneity enhancement) were independent predictors of malignancy. The CMR mass score (range, 0-8 and cutoff, ≥5), including sessile appearance, polylobate shape, infiltration, pericardial effusion, first-pass contrast perfusion, and heterogeneity enhancement, showed excellent accuracy in predicting malignancy (areas under the curve, 0.976 [95% CI, 0.96-0.99]), significantly higher than diagnostic echocardiographic mass score (areas under the curve, 0.932; P=0.040). The agreement between the diagnostic echocardiographic mass and CMR mass scores was good (κ=0.66). A CMR mass score of ≥5 predicted a higher risk of all-cause death (P<0.001; hazard ratio, 5.70) at follow-up.

A CMR-derived model, including mass morphology and tissue characterization, showed excellent accuracy, superior to echocardiography, in predicting cardiac masses malignancy, with prognostic implications.

Cardiac masses are a significant cause of patient morbidity and mortality by virtue of their symptoms and surgical removal. Preoperative diagnosis of a cardiac mass is usually based on clinical correlation and transthoracic echocardiography findings. Myxomas are the most common benign cardiac tumors, commonly occurring in the left atrium attached to the interatrial septum near the fossa ovalis. Although, at times atypical location and unusual morphology may pose a diagnostic dilemma with 2D echocardiography. 3D echocardiography with its multifaceted advantages, including multiplanar cropping abilities and superior imaging quality can help distinguish between a clot and a myxoma.

Heart masses, including tumors (primary and secondary) and pseudotumor (cysts and thrombus), are rare entities, but of increasing interest in cardiac imaging areas. The clinical manifestations are related to the intracardiac effect of mass, embolization, and systemic symptoms in the case of tumors; however, some of them are detected incidentally. Nowadays, imaging techniques and the advancement of their tools perform the morphological, functional, and tissue characterization of the masses, and additionally know the anatomical relationships, which are crucial factors for the treatment and surgical planning.

The use of myocardial perfusion imaging during a stress cardiac magnetic resonance (CMR) examination for the evaluation of coronary artery disease is now recommended by both US and European guidelines. Several studies have demonstrated high diagnostic accuracy for the detection of hemodynamically significant coronary artery disease. Stress perfusion CMR has been shown to be a noninvasive and cost-effective alternative to guide coronary revascularization.

Primary intimal sarcomas of the heart are extremely rare and have a dismal prognosis. Their management represents a complex clinical challenge since complete surgical resection is the only reliable possibility of cure but is only possible in 50% of patients. In non-resectable disease, anthracycline-based therapy is the most effective treatment, but pazopanib may be used in patients unfit to receive anthracyclines.

A 38-year-old man presented with acute right heart failure symptoms due to a primary intimal sarcoma of the heart. A definite diagnosis was made after cardiac surgery. Multi-modality cardiac imaging showed early recurrence of disease with mitral valve and pulmonary veins' invasion, and the patient was deemed inoperable. Due to chronic kidney disease and previous heart failure symptoms, he was started on first-line pazopanib palliative treatment. After 11 months of chemotherapy, there was good clinical tolerance and no evidence of disease progression, which occurred after 13 months.

This case highlights the value of a multi-modality imaging approach for cardiac masses. Most importantly, it reports the successful treatment of a young patient with a primary intimal sarcoma of the heart who was started on palliative pazopanib, with a significantly higher progression-free survival than is reported in the literature. This finding may support pazopanib as a good alternative as first-line treatment when there is contraindication for anthracycline-based chemotherapy.

Nonbacterial thrombotic endocarditis (NBTE) is a distinctive condition marked by the presence of aseptic fibrin depositions on cardiac valves due to hypercoagulability and endocardial damage. There is a scarcity of large cohort studies clarifying factors associated with morbidity and mortality of this condition. A systematic literature review was performed utilizing the PubMed, Embase, Cochrane, and Web-of-Science databases to retrieve case reports and series documenting cases of NBTE from inception until September-2022. A descriptive analysis of basic characteristics was carried out, followed by multivariate regression analysis to identify risk factors associated with morbidity and mortality. A total of 416 case reports and series were identified, of which 450 patients were extracted. The female-to-male ratio was around 2:1 with an overall sample median age of 48 (interquartile range [IQR]:34-61). Stroke-like symptoms were the most common presentation and embolic phenomena occurred in 70% of cases, the majority of which were due to stroke. Cancer was associated with higher embolic complications (aOR:6.38, 95% CI = 3.75-10.83, p < 0.01) in comparison to other NBTE etiologies, while age, sex, and vegetation size were not (p > 0.05). All-cause in-hospital mortality was 36%, with cancer etiology being associated with higher mortality: 56% (aOR:3.64, 95% CI = 1.57-8.43, p < 0.01) in comparison to other NBTE etiologies:19%. A significant decrease in NBTE mortality was seen in recent years in comparison to admissions that occurred during the 20th century (aOR:0.07, 95% CI = 0.04-0.15, p < 0.01). While there has been an observed improvement in overall in-hospital mortality rates for patients admitted with NBTE in recent years, it is important to note that cases associated with a cancer etiology are still linked to high morbidity and mortality during hospitalization.

A cardiac lesion detected at ultrasonography might turn out to be a normal structure, a benign tumor or rarely a malignancy, and lesion characterization is very important to appropriately manage the lesion itself. The exact relationship of the mass with coronary arteries and the knowledge of possible concomitant coronary artery disease are necessary preoperative information. Moreover, the increasingly performed coronary CT angiography to evaluate non-invasively coronary artery disease leads to a rising number of incidental findings. Therefore, CT and MRI are frequently performed imaging modalities when echocardiography is deemed insufficient to evaluate a lesion. A brief comprehensive overview about diagnostic radiological imaging and the clinical background of cardiac masses and pseudomasses is reported.

"Cases of SCMR" is a case series on the SCMR website (https://www.scmr.org) for the purpose of education. The cases reflect the clinical presentation, and the use of cardiovascular magnetic resonance (CMR) in the diagnosis and management of cardiovascular disease. The 2022 digital collection of cases are presented in this manuscript.

The gold standard for diagnosis of cardiac tumours is histopathological examination. Cardiovascular magnetic resonance (CMR) is a valuable non-invasive, radiation-free tool for identifying and characterizing cardiac tumours. Our aim is to understand CMR diagnosis of cardiac tumours by distinguishing benign vs. malignant tumours compared to the gold standard.

A systematic search was performed in the PubMed, Web of Science, and Scopus databases up to December 2022, and the results were reviewed by 2 independent investigators. Studies reporting CMR diagnosis were included in a meta-analysis, and pooled measures were obtained. The risk of bias was assessed using the Quality Assessment Tools from the National Institutes of Health.

A total of 2,321 results was obtained; 10 studies were eligible, including one identified by citation search. Eight studies were included in the meta-analysis, which presented a pooled sensitivity of 93% and specificity of 94%, a diagnostic odds ratio of 185, and an area under the curve of 0.98 for CMR diagnosis of benign vs. malignant tumours. Additionally, 4 studies evaluated whether CMR diagnosis of cardiac tumours matched specific histopathological subtypes, with 73.6% achieving the correct diagnosis.

To the best of our knowledge, this is the first published systematic review on CMR diagnosis of cardiac tumours. Compared to histopathological results, the ability to discriminate benign from malignant tumours was good but not outstanding. However, significant heterogeneity may have had an impact on our findings.

Primary cardiac and pericardial neoplasms are rare in the pediatric population and can include both benign and malignant lesions. Rhabdomyomas, teratomas, fibromas, and hemangiomas are the most common benign tumors. The most common primary cardiac malignancies are soft-tissue sarcomas, including undifferentiated sarcomas, rhabdomyosarcomas, and fibrosarcomas. However, metastatic lesions are more common than primary cardiac neoplasms. Children with primary cardiac and pericardial tumors may present with nonspecific cardiovascular symptoms, and their clinical presentation may mimic that of more common nonneoplastic cardiac disease. The diagnosis of cardiac tumors has recently been facilitated using noninvasive cardiac imaging. Echocardiography is generally the first-line modality for evaluation. Cardiac MRI and CT are used for tissue characterization and evaluation of tumor size, extension, and physiologic effect. The varied imaging appearances of primary cardiac neoplasms can be explained by their underlying abnormality. Treatment of these lesions varies from conservative management, with spontaneous regression of some lesions such as rhabdomyomas, to surgical resection, particularly in patients with associated heart failure. With adequate imaging techniques and knowledge of the pathologic basis of the neoplasm, it is often possible to differentiate benign from malignant tumors, which can greatly affect adequate and timely treatment. ©RSNA, 2023 Quiz questions for this article are available through the Online Learning Center.

Melanoma is considered a masquerader of many diseases owing to its potential to metastasize to many organs. Several malignancies can metastasize to the heart including malignant melanoma. Historically, antemortem diagnosis of cardiac involvement of melanoma is not common, but with significant improvement in imaging modalities, the diagnosis can now be made early and accurately, aiding in treatment and improved survival. We present a case of a 36-year-old man with brief neurological symptoms and subsequent diagnosis of cerebrovascular accident (CVA). Cardiac imaging revealed incidental findings of right and left ventricular masses and lymph node biopsy, confirming metastatic melanoma. Cardioembolic etiology was suspected for his CVA. Prompt immunotherapy was initiated with improvement in his clinical condition.

Primary cardiac tumors are rare in all ages, especially in children, with a reported prevalence range of 0.0017-0.28% in autopsy series. Due to their rarity, the diagnostic and therapeutic pathways reserved to them are usually described by single case reports, leading to the point where a common diagnostic protocol is imperative to obtain a differential diagnosis. The first diagnostic approach is done with transthoracic echocardiogram (TTE), due to its wide availability, low cost, absence of ionizing radiations and non-invasiveness. Several tumors are discovered incidentally and, in many cases, TTE is helpful to determine location, size and anatomical features, playing a key role in the differential diagnosis. In the last few years, cardiac magnetic resonance imaging (CMR) has had an increased role in the diagnostic pathway of pediatric cardiac masses, due to its high accuracy in characterizing mass tissue properties (especially for soft tissue), and in detecting tumor size, extent, pericardial/pleural effusion, leading to the correct diagnosis, treatment and follow-up. Therefore, nowadays, several consensus statements consider CMR as a leading imaging technique, thanks to its non-invasive tissue characterization, without the use of ionizing radiation, in an unrestricted field of view. As suggested by the most recent literature, the pediatric protocol is not so different from the adult one, adapted to the size and cardiac frequency of the patient, sometimes requiring special conditions such as free-breathing sequences and/or sedation or general anesthesia in non-cooperating patients.

Primary pericardial sarcomas are rare and lethal diseases. To date, only a few cases of primary pericardial sarcomas, such as rhabdomyosarcoma (RMS), have been reported. Since the unusual location of RMS in the pericardium makes it challenging to diagnose, precise diagnostic procedures are required. In this study, we present the case of a 23-year-old man who experienced postprandial obstruction and atypical precordial pain that lasted for a week. Echocardiography revealed a heterogeneous isoechoic pericardial mass with a significant pericardial effusion. Contrast-enhanced CT revealed a massive pericardial effusion along with an irregular, defined, heterogeneously enhancing mass that was located between the pericardium and diaphragm. PET-CT imaging showed an intense FDG uptake in the pericardial mass. Furthermore, cardiac MRI demonstrated malignant characteristics of the pericardial mass and provided a detailed visualization of its exact anatomical connection with both cardiac and extracardiac structures. Finally, a pathologic examination of a puncture biopsy specimen confirmed the diagnosis of primary pericardial RMS. Our case emphasizes the importance of multimodal imaging for the differential diagnosis and evaluation of cardiac involvement, while providing clinicians with crucial information for clinical treatment and decision-making.

Cardiac magnetic resonance (CMR) imaging has been established as a valuable diagnostic tool in the assessment of pericardial diseases by providing information on cardiac anatomy and function, surrounding extra-cardiac structures, pericardial thickening and effusion, characterization of pericardial effusion, and the presence of active pericardial inflammation from the same scan. In addition, CMR imaging has excellent diagnostic accuracy for the non-invasive detection of constrictive physiology evading the need for invasive catheterization in most instances. Growing evidence in the field suggests that pericardial enhancement on CMR is not only diagnostic of pericarditis but also has prognostic value for pericarditis recurrence, although such evidence is derived from small patient cohorts. CMR findings could also be used to guide treatment de-escalation or up-titration in recurrent pericarditis and selecting patients most likely to benefit from novel treatments such as anakinra and rilonacept. This article is an overview of the CMR applications in pericardial syndromes as a primer for reporting physicians. We sought to provide a summary of the clinical protocols used and an interpretation of the major CMR findings in the setting of pericardial diseases. We also discuss points that are less well clear and delineate the strengths and weak points of CMR in pericardial diseases.

Primary cardiac tumors account for only 0.3% of all cardiac tumors; of these, lymphomas account for only 2% of all primary cardiac tumors. Cardiac lymphomas have a grim prognosis, often less than 1 year due to delays in diagnosis and treatment. Cardiac MRI is the gold standard for the imaging of cardiac tumors. We describe the case of a 76-year-old man with no significant past medical history who presented to the emergency department with a large pericardial effusion that was found to be consistent with cardiac lymphoma on cardiac MRI prior to tissue diagnosis of a primary cardiac diffuse large B-cell lymphoma. The clinical and radiological features of cardiac lymphoma are reviewed, and the therapeutic management and side-effects that the patient experienced are discussed.

There is a paucity of quantitative measurements of cardiac tumors and myocardium using parametric mapping techniques. This study aims to explore quantitative characteristics and diagnostic performance of native T1, T2, and extracellular volume (ECV) values of cardiac tumors and left ventricular (LV) myocardium.

Patients with suspected cardiac tumors who underwent cardiovascular magnetic resonance (CMR) between November 2013 and March 2021 were prospectively enrolled. The diagnoses of primary benign or malignant tumors were based on pathologic findings if available, comprehensive medical history evaluations, imaging, and long-term follow-up data. Patients with pseudo-tumors, cardiac metastasis, primary cardiac diseases, and prior radiotherapy or chemotherapy were excluded. Multiparametric mapping values were measured on both cardiac tumors and the LV myocardium. Statistical analyses were performed using independent-samples t-test, receiver operating characteristic, and Bland-Altman analyses.

A total of 80 patients diagnosed with benign (n = 54), or primary malignant cardiac tumors (n = 26), and 50 age and sex-matched healthy volunteers were included. Intergroup differences in the T1 and T2 values of cardiac tumors were not significant, however, patients with primary malignant cardiac tumors showed significantly higher mean myocardial T1 values (1360 ± 61.4 ms) compared with patients with benign tumors (1259.7 ± 46.2 ms), and normal controls (1206 ± 44.0 ms, all P < 0.05) at 3 T. Patients with primary malignant cardiac tumors also showed significantly higher mean ECV (34.6 ± 5.2%) compared with patients with benign (30.0 ± 2.5%) tumors, and normal controls (27.3 ± 3.0%, all P < 0.05). For the differentiation between primary malignant and benign cardiac tumors, the mean myocardial native T1 value showed the highest efficacy (AUC: 0.919, cutoff value: 1300 ms) compared with mean ECV (AUC: 0.817) and T2 (AUC: 0.619) values.

Native T1 and T2 of cardiac tumors showed high heterogeneity, while myocardial native T1 values in primary malignant cardiac tumors were elevated compared to patients with benign cardiac tumors, which may serve as a new imaging marker for primary malignant cardiac tumors.