Approach to cardiac masses: Thinking inside and outside the box.

In this editorial we comment on the article by Huffaker et al, published in the current issue of the World Journal of Clinical Cases. Cardiac masses encompass a broad range of lesions, potentially involving any cardiac structure, and they can be either neoplastic or non-neoplastic. Primitive cardiac tumors are rare, while metastases and pseudotumors are relatively common. Cardiac masses frequently pose significant diagnostic and therapeutic challenges. Multimodality imaging is fundamental for differential diagnosis, treatment, and surgical planning. In particular cardiac magnetic resonance (CMR) is currently the gold standard for noninvasive tissue characterization. CMR allows evaluation of the relationship between the tumor and adjacent structures, detection of the degree of infiltration or expansion of the mass, and prediction of the possible malignancy of a mass with a high accuracy. Different flow charts of diagnostic work-up have been proposed, based on clinical, laboratory and imaging findings, with the aim of helping physicians approach the problem in a pragmatic way ("thinking inside the box"). However, the clinical complexity of cancer patients, in particular those with rare syndromes, requires a multidisciplinary approach and an open mind to go beyond flow charts and diagnostic algorithms, in other words the ability to "think outside the box".

Report of a giant invasive, wall-penetrating cardiac lipoma.

BACKGROUND: Cardiac lipoma, a seldom-encountered benign tumor positioned beneath the endocardium, has the potential to impair electrophysiological functions. Diagnosis is principally based on imaging modalities. The uniqueness of this case lies in the tumor's extension both internally and externally within the right atrium, rendering it of special interest. From a clinical standpoint, surgical removal is commonly advocated, wherein early intervention is pivotal in improving patients' long-term prognoses. CASE PRESENTATION: A 35-year-old male was admitted to the hospital for treatment subsequent to the identification of a cardiac mass two days prior. Initial diagnostic assessments, encompassing CT scans and echocardiography, identified a space-occupying lesion within the heart. The patient underwent surgical excision of the cardiac tumor, utilizing mild hypothermic extracorporeal circulation via femoral vessel access. Intraoperative findings revealed adipose-like tissue of a "dumbbell-shaped" configuration situated both within and external to the right atrium, measuring approximately 8 cm*9 cm internally and 7 cm*6 cm externally, with the extracardiac mass being marginally larger. Postoperative pathological analysis confirmed a cardiac lipoma diagnosis. A follow-up echocardiogram conducted three months post-surgery exhibited no notable abnormalities. The patient is under continuous observation to monitor for any recurrence or potential long-term complications. CONCLUSION: In this case report, we detail with precision a rare cardiac pathology manifested by an expansive infiltrative lipoma that pervades the endocardial and epicardial layers of the right atrium. After thorough preoperative diagnostic workup and evaluation, we contend that surgical intervention represents the optimal therapeutic approach for managing such conditions, with the goal of preemptively reducing the likelihood of cardiac compression or intracardiac obstruction.

Surgical planning aided with 3D technologies for management of complex paracardiac tumors.

BACKGROUND: Accurate diagnosis and treatment of complex cardiac tumors poses challenges, particularly when surgical resection is considered. 3D reconstruction and printing appear as a novel approach to allow heart teams for optimal surgical and post operative care. METHODS: We report two patients with uncommon masses including a cardiac angiosarcoma (CAS) and a IgG4-related disease (IgG4-RD) with exclusive cardiac involvement. In both cases, three-dimensional (3D) reconstruction and 3D-printed models were utilized to aid the surgical team achieve optimal pre-operative planning. Both patients underwent ECG-gated cardiac computed tomography angiography (CCTA) imaging and, due to the complex anatomy of the masses, their large dimensions, proximity to vital cardiac and vascular structures, and unclear etiology, computational and 3D-printed models were created for surgical planning. An exploratory literature review of studies using 3D-printed models in surgical planning was performed. RESULTS: In case 1 (CAS), due to the size and extension of the mass to the right ventricular free wall, surgical intervention was not considered curative and, during thoracotomy, an open biopsy confirmed the imaging suspicion of CAS which guided the initiation of optimal medical treatment with chemotherapy and, after clear tumor retraction, the patient underwent a second surgical intervention, and during the 18 months of follow-up showed no signs of recurrence. In Case 2 (IgG4-RD), the patient underwent uncomplicated total surgical resection; this allowed directed treatment and, at 12 months follow-up, there are no signs of recurrence. Computational and 3D-printed models were used to plan the surgery and to confirm the findings. Limited studies have explored the use of 3D printing in the surgical planning of tumors. CONCLUSIONS: We present two patients with uncommon cardiac tumors, highlighting the significant value of 3D models in the anatomical characterization and assessment of their extension. These models may be essential in surgical planning for complex cardiovascular cases and could provide more information than conventional imaging modalities. Further studies are needed to demonstrate the impact of 3D technologies in studying cardiac tumors.

Cardiac Hepatoma: An Unlikely Cause of Malignant Cardiac Metastatic Disease.

Cardiac hepatoma is an extremely rare presentation of tumor metastasis. We present a case of an 80-year-old male who presented with symptoms of heart failure and was subsequently diagnosed with cardiac metastatic tumors. This case report highlights the diagnostic challenges and management options associated with this rare entity.

Echocardiography-Guided Radiofrequency Ablation for Cardiac Tumors.

Background: Patients with cardiac tumors may present challenges for surgical resection due to poor clinical condition. Echocardiography-guided transapical radiofrequency ablation for cardiac tumors (TARFACT) potentially offers a less invasive palliative therapy option. Objectives: This study aimed to evaluate the safety and efficacy of TARFACT. Methods: Five patients with cardiac tumors (mucinous liposarcoma, myocardial hypertrophy with inflammatory cell infiltration mass, fibrous tissue tumor hyperplasia, myocardial clear cell sarcoma, and cardiac rhabdomyoma) were included. All patients underwent TARFACT and were assessed with electrocardiogram, echocardiographic imaging, biochemical analysis, and pathological confirmation. Results: The median follow-up for all patients was 9 (range 4-12) months. Three surviving patients were alive at their last follow-up (9, 12, and 12 months, respectively), whereas 2 patients with late-stage tumors survived 6 months and 13 months after TARFACT, respectively. After TARFACT, all patients showed significant reductions in tumor size: the mean length decreased from 6.7 ± 2.0 cm to 4.7 ± 1.8 cm (P = 0.007); and the mean width decreased from 5.0 ± 2.1 cm to 2.5 ± 0.7 cm (P = 0.041). NYHA functional class also improved: median (IQR) decreased from 3.0 (1.5) to 2.0 (1.0) (P = 0.038), Peak E-wave on echocardiography showed a mean increase from 64.4 ± 15.7 cm/s to 76.6 ± 18.6 cm/s (P = 0.008), and NT-pro BNP levels had a median (IQR) reduction from 115.7 (252.1) pg/mL to 55.0 (121.6) pg/mL (P = 0.043). Conclusions: TARFACT is a novel palliative treatment option for cardiac tumors, reducing accessible tumors and improving clinical symptoms in a preliminary group of patients. (Cardiac Tumors Interventional [Radio Frequency/Laser Ablation] Therapy [CTIH]; NCT02815553).

Type and dimensions can predict ventricular arrhythmias and cardiac death in primary benign cardiac tumors in children.

Despite being extremely rare in children, primary benign cardiac tumors can cause malignant ventricular arrhythmias (VA) or even sudden cardiac death. To assess the predictors of cardiovascular death and malignant VAs, we designed a retrospective single-center study enrolling paediatric patients. We defined as primary outcome a composite of cardiovascular death, sustained VT, ventricular fibrillation and rapid, symptomatic non-sustained VT. Our secondary endpoint was to assess the prevalence of clinically significant arrhythmias in our population. METHODS AND RESULTS: We fitted a multivariate Cox regression model to assess the predictors of the primary outcome. Over a period of 38 years, a total of 97 children were enrolled in the study. Among them, there were 73 rhabdomyomas, 13 fibromas, 3 myxomas, 3 teratomas, 1 lipoma, 2 haemangiomas and 2 fibroelastomas. Over a median follow up of 10.53 years, 16 patients met the primary outcome. Kaplan Meier unadjusted survival estimates showed that tumor dimensions larger than 2.3 cm and diagnosis of fibroma predicted worse outcomes compared with smaller tumors or other histotypes, (log rank p < 0.0002 and < 0.0001 respectively). In multivariate Cox proportional hazards analysis, diagnosis of fibroma and tumor dimensions were independently associated to the primary endpoint (HR: 5.06, 95 %CI (1.3-19); and 1.26 • (1.05-11), respectively). Clinically significant arrhythmias were reported in 24.5 % of the study population. CONCLUSIONS: Among paediatric primary cardiac tumors, type and dimensions may predict the hazard of malignant VAs and cardiac death.

Cardiac Paraganglioma in a Young Patient Presents with Angina-like Symptoms: A Case Report and Literature Review.

Paragangliomas are rare extra-adrenal neuroendocrine tumors originating from chromaffin tissue that present a diagnostic and therapeutic challenge due to their diverse clinical manifestations and low incidence. While these tumors often manifest as catecholamine-secreting functional tumors, their clinical presentation can vary, leading to delayed diagnosis and challenging management. This study presents the case of a 22-year-old patient with cardiac paraganglioma who initially presented with angina-like symptoms, highlighting the importance of considering this rare condition in young individuals with nonspecific complaints. Diagnostic imaging, including transthoracic echocardiography, CT angiography, and MRI, played a crucial role in identifying the tumor's location and vascularization. Surgical excision, including pulmonary artery graft and CABG, was the primary management approach, which was accompanied by intraoperative complications that later led to CCU admission, followed by postoperative complications, ultimately leading to the patient's death. This case highlights the significance of early recognition and management of complications following a surgical approach to treat paragangliomas.

Metastatic choriocarcinoma to the heart and lung with successful multimodal management.

Cardiac tumors continue to be one of the most uncommon primary tumors. Secondary cardiac tumors are rare and occur in difficult instances. A multidisciplinary team is required for diagnosis and management. Within this case study, we present a 24-year-old female patient who experienced dyspnea and was diagnosed as having metastatic heart and lung choriocarcinoma that was effectively managed with heart surgery and chemotherapy.

A case report on asymptomatic ventricular hemangioma.

Intraventricular cardiac hemangiomas are very rare tumors that account for 5-10% of all cardiac tumors. We report a case of an asymptomatic 21-year-old young male who was incidentally detected with ventricular mass on echocardiography followed by magnetic resonance imaging. Surgical resection was done for confirmation and to avoid potential complications. Histopathology and immunohistochemical studies confirmed the diagnosis of a hemangioma of the ventricle. Subsequent regular follow-up has revealed no complications or recurrence, underscoring the favorable long-term prognosis. This case report highlights the importance of diagnostic evaluation even in asymptomatic individuals, allowing for early recognition and effective management of this rare cardiac entity.

Confounding giant right atrial mass.

BACKGROUND: Primary cardiac tumors are uncommon, with approximately 70-80% classified as benign. Myxomas constitute roughly half of all benign cardiac tumors, while cardiac hydatid cysts are exceptionally rare. Shortness of breath is a prominent symptom associated with these conditions. Echocardiography serves as the primary diagnostic tool, enabling early detection. The preferred course of action involves the surgical excision of the mass. CASE PRESENTATION: Our first case, a 51-year-old female presented to the cardiology outpatient clinic with complaints of shortness of breath and palpitations persisting for 3 months. Physical examination and tests were within normal limits. Echocardiography revealed a right atrial myxoma measuring 65 * 35 mm. Despite not affecting valve and ventricular functions, the mass was surgically resected. Our second case, a 55-year-old male admitted to the cardiology clinic with recurrent fever, shortness of breath, and chest pain. Physical examination and tests were normal. Echocardiography revealed an echogenic mass with a septate appearance adhering to the free wall of the right atrium. The patient, diagnosed with a hydatid cyst, underwent surgical resection. CONCLUSIONS: We came across two different cases of RA masses which happened to be myxoma and hydatid cysts, and we managed accordingly. The right atrial myxomas typically present with nonspecific shortness of breath unless they cause valve obstruction. In cases where clinical findings suggest infection in the right atrial masses, it is essential to consider hydatid cyst as a potential diagnosis alongside the initial consideration of vegetation.

Additional value of CMR parametric mapping in tissue characterization of common benign pediatric cardiac tumors.

BACKGROUND: Cardiac Magnetic Resonance (CMR) parametric mapping is underexplored in cardiac tumors. OBJECTIVES: To evaluate the contribution of mapping sequences on the characterization of pediatric tumors. METHODS: All pediatric patients referred for cardiac tumors at Bambino Gesù Children's Hospital from June 2017 to November 2023, who underwent CMR with mapping sequences, were included. The diagnosis of tumor type was performed according to signal characteristics on different sequences. Mass parametric mapping for each subtype and interobserver variability was assessed. RESULTS: Sixteen patients were enrolled. The mean age at CMR was 7 ± 5 years. "Traditional" mass-type assessment diagnosed hemangioma (Group A) in 3 patients (19%), fibroma (Group B) in 4 patients (25%), rhabdomyoma (Group C) in 6 patients (37%), and lipoma (Group D) in 3 patients (19%). The ANOVA 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 msec and 54 ± 4% for Group A, 860 ± 118 msec and 93 ± 4% for Group B, 1007 ± 57 msec and 23 ± 5% for Group C, and 215 ± 13 msec and 0 ± 0% for Group D. CONCLUSIONS: 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 hemangioma.

Cardiac rhabdomyoma: an uncommon culprit in sudden infant death.

Sudden death in infants remains a common and poorly understood cause of childhood mortality in the USA. Pediatric cardiac tumors, although rare, may underlie some cases of unexplained sudden infant death. Autopsy is a crucial diagnostic step in these cases, as both gross and microscopic examination of the heart may uncover occult cardiac tumors. Rhabdomyomas are the most common cardiac tumors in childhood and may result in arrythmia and sudden death. We present a case of sudden death in a healthy 5-month-old infant which initially appeared "SIDS-like" until thorough histological analysis revealed an underlying cardiac rhabdomyoma. The case is of particular importance in that the gross examination of the heart was considered completely normal, and the tumor only involved certain portions of the heart microscopically. Had a single random section of myocardium been the only heart section examined microscopically, the diagnosis might have been missed. This case emphasizes the importance of thorough microscopic examination in infant cases, especially in cases where the heart appears grossly normal.

STEMI or not STEMI? A multimodality imaging approach to a challenging intracardiac mass with a tricky presentation.

Cardiac tumors, due to the various clinical scenarios and their histological subtypes, are still challenging for clinicians. They are differentiated into primary and secondary. The latest are more common and are usually lung and breast cancers, melanomas, and lymphoma metastasis. We present a case of a 73-year-old woman, with a history of breast cancer 10 years earlier, admitted to Cath lab for an elevation of the ST-segment of the electrocardiogram, myocardial infarction. Echocardiogram showed a curious abnormality in the myocardial wall. Thanks to a multimodality imaging strategy, including contrast-enhanced echocardiography and cardiac magnetic resonance, characterization of the underlying pathology was clear and, thus, the appropriate management and therapy.

This is the case of a cardiac metastatic tumor, whose certain diagnosis can only be made by myocardial biopsy ­ an invasive sample of heart tissue ­ unfortunately not available in our case. Alternatively, we puzzled data from two second-level imaging techniques: contrast-enhanced cardiac ultrasound and cardiac magnetic resonance, which allowed us to better evaluate the nature of this cardiac mass. The former gave information about its blood supply, the latter gave information about tissue characterization. In this paper, we show how a complete integration of data from clinical and a stepwise multimodality imaging-based approach may allow a diagnosis in a complex clinical case.

Myxoma Involving the Pulmonary Valve - A Case Report of an Atypical Cardiac Tumor, Treated Using an Unusual Approach.

Background: Atypical myxoma has been reported in various locations in the heart, however, myxoma involving the pulmonary valve is rare. Here we present a case of pulmonic valve myxoma which was resected via a percutaneous approach. Case Report: A 66-year-old female with known metastatic adenocarcinoma of the lung, and chronic obstructive pulmonary disease presented with acute onset shortness of breath for two days. The patient experienced respiratory arrest en-route to the hospital and required intubation. Computed tomography angiography (CTA) of the chest revealed a new 1.4 × 1.6 cm intracardiac mass along the pulmonary valve. Further evaluation with cardiac magnetic resonance imaging revealed it to be a large vascular tumor on the ventricular side of the pulmonary valve, attached with a narrow stalk. Due to high surgical risk, the patient underwent transesophageal echocardiographic guided percutaneous removal of the mass. Pathology confirmed the mass to be a myxoma. Conclusion: Atypical myxoma should be considered in the differential diagnosis of valvular masses. Percutaneous resection of valvular masses may be feasible in high-risk surgical patients. LEARNING POINTS: Pulmonary valve myxoma is a rare condition and the literature on the characteristics and treatment options for pulmonary valve myxoma is limited.Our patient was treated with a minimally invasive treatment approach: removal of a tumor with intra operative transesophageal echocardiographic guidance using AngioVac and Flow Triever catheters.Percutaneous resection of valvular masses may be feasible in high surgical risk patients.

Congenital Blood Cyst of a Child: A case report and review of literature.

Blood-filled cysts of the heart valves are frequently reported at postpartum autopsies of infants. They are seen as round nodules mostly in the paediatric age group in infants less than 2 months of age and disappear spontaneously within 6 months of life. We report a unique case of an 11-month-old girl who presented at a tertiary healthcare hospital in 2022 with a blood-filled cyst on the posterior leaflet of the pulmonary valve that was successfully treated. This case report highlights the characteristics and course of a paediatric patient with blood-filled cysts. Further studies are yet needed to better understand the diagnostic approaches to blood-filled cysts as well as treatment modalities to fill the gap in clinical settings.

A case of left atrial intimal sarcoma with rhabdomyosarcoma differentiation: a case report and literature review.

Primary cardiac malignancies are rare, with cardiac sarcomas being the main type. Among these, intimal sarcomas are the most common. However, they tend to occur in the great vessels and are rare in the heart, with only a few isolated cases reported. We report a challenging case of a patient with left atrial intimal sarcoma with rhabdomyosarcoma differentiation. The patient was admitted after a physical examination detected left heart occupancy, and initial imaging suspected a left atrial thrombus. The patient then underwent extracorporeal circulation-assisted open cardiac surgery with resection of an atrial mass. The postoperative pathological findings were suggestive of an arterial intimal sarcoma, which included areas of rhabdomyosarcoma differentiation within the tumor tissue. Unfortunately, the patient's tumor recurred 4 months later, and she died due to treatment failure. This case highlights the rarity and risk of misdiagnosis of cardiac intimal sarcoma. Additionally, we aim to improve the understanding of intimal sarcoma through a review of immunohistochemistry and gene amplification techniques.

Primary intracardiac germ cell tumor.

We present the echocardiography images in a 6 year old girl who presented with a history of scalp swelling after trivial trauma which was subsequently diagnosed as metastases from primary intracardiac germ cell tumour.

Cardiac solitary fibrous tumor - an extremely rare but potentially fatal diagnosis.

The background of this review is a description of the case of a 28-year-old man with an extremely rare cardiac solitary fibrous tumor (SFT). Although this tumor was removed surgically and in the 6-month follow-up examination no relapse was noted, recurrence was observed and confirmed in the magnetic resonance imaging 4 months later. SFT prevalence, symptoms and signs, treatment options and prognosis are reviewed.

Rheumatic heart disease of the mitral valve alongside the papillary fibroelastoma of the aortic valve: A case report.

Key Clinical Message: Besides thromboses, it's crucial to also consider the rare possibility of tumors like papillary fibroelastomas when evaluating worsening cardiopulmonary symptoms in patients with severe rheumatic mitral stenosis and atrial fibrillation. Abstract: Cardiac papillary fibroelastoma is a rare and benign endocardial tumor typically found on the aortic valve. The simultaneous occurrence of rheumatic heart disease affecting the mitral valve and papillary fibroelastoma on the aortic valve is infrequent, with limited documented instances. This unique case can enhance our understanding of the clinical presentation, diagnostic approaches, management options, and implications for patient outcomes in these two conditions. We present the case of a 47-year-old woman who was admitted to the hospital due to worsening dyspnea and fatigue, during which time she discovered an aortic valve papillary fibroelastoma. Further investigations revealed two thrombi in her left atrium and left atrial appendage, along with significant rheumatic mitral valve stenosis. The patient underwent thrombectomy, mitral valve replacement, and valve-sparing aortic valve tumor resection. Following surgery, the patient's recovery was unremarkable. This case report emphasizes the need for a comprehensive evaluation in patients with rheumatic mitral stenosis, considering all possible etiologies. While thrombi are typical in mitral stenosis and atrial fibrillation, the rare presence of tumors like papillary fibroelastomas should be recognized, underscoring the importance of further assessment when suspicion arises. Importantly, individuals with asymptomatic cardiac papillary fibroelastomas should undergo surgical treatment to minimize the potential risk of tumoral embolization.