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Papillary Fibroelastoma

Papillary Fibroelastoma

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Cardiac masses are rare, but remain an important component of cardio-oncology practice. These include benign tumors, malignant tumors (primary and secondary) and tumor-like conditions (e.g., thrombus, Lambl’s excrescences, and pericardial cyst). The advent of multimodality imaging has enabled identification of the etiology of cardiac masses in many...

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... The stalk-like lesion identified on TEE was initially concerning for an atypical thrombus or an atrial myxoma. Typically, myxomas are mobile masses with stalk-like attachments to the fossa ovalis or mitral annulus, but they can be located throughout the endocardium [5]. Thrombi are avascular and do not typically enhance on CMR. ...
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Atrial fibrillation, the most common cardiac arrhythmia in the Western world, confers a 5-fold increase in stroke, mainly due to thrombus formation in the left atrial appendage. Early rhythm control is often beneficial in reducing adverse cardiovascular events in higher-risk populations. Here, we present a patient who was found to have a 1 cm stalk-like lesion in the left atrial appendage on transesophageal echocardiogram prior to electrical cardioversion. Using multiple cardiac imaging modalities, including cardiac magnetic resonance imaging and computed tomography, the mass was eventually determined to be a chronic resolving thrombus.
... Cardiac myxomas represent the most common primary cardiac tumors, accounting for approximately 50% of all benign heart tumors. Originating primarily in the atrial chambers, their clinical presentations vary extensively, often leading to misdiagnosis or late diagnoses [16]. ...
... However, echocardiographic findings must always be interpreted in the clinical context of the patient and alternative diagnoses should be considered. Table 3 displays the differences between the main causes of intracardiac masses [27,28]. ...
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Point-of-Care Ultrasound (POCUS) is a safe, non-invasive technique performed at the patient's bedside, providing immediate results to the operator. It complements physical examination and facilitates clinical decision-making. In infectious diseases, POCUS is particularly valuable, offering an initial assessment in cases of suspected infection. It often leads to an early tentative diagnosis enabling the prompt initiation of antimicrobial treatment without the delay associated with traditional radiology. POCUS provides direct visualization of affected organs, assists in evaluating fluid balance and facilitates various interventions, all while reducing patient discomfort. For infectious disease specialists, becoming proficient in POCUS is a critical future challenge, requiring dedicated training for effective utilization.
... Primary cardiac lymphomas (PCLs) are very rare malignancies, concerning only 1.3% of already-rare primary cardiac tumors (PCTs) [1]. Because of the risk for myocardial rupture, the potential benefits of chemotherapy must be balanced against the risk of cardiotoxicity. ...
... Herein, we present a unique case of a 75-year-old male, diagnosed with primary cardiac diffuse large B-cell lymphoma (DLBCL) with relatively unusual involvement of the coronary sinus, treated first with one cycle of R-EPOCH, followed by three cycles of R-CHOP to reduce said risk. To our knowledge, Aryan Rezvani aryanrezvani@icloud.com 1 in the infero-medial RA, extending within the coronary sinus, measuring 4.4 × 8.3 cm, with a maximum SUV of approximately 16. There was a 5 mm lung nodule in the left lower lobe of the lung with a maximum SUV of 2.2 as well, suggestive of metastasis. ...
... PCLs are very rare malignancies, in which a lymphomatous tumor primarily involves the heart [3]. The most common type of lymphoma involved is DLBCL, representing greater than or equal to 80% of PCLs [3], but others have been described, including Burkitt lymphoma, low-grade B-cell lymphoma, and T-cell lymphoma [1,3]. ...
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Primary cardiac lymphomas (PCLs) are a rare clinical entity, in which treatment guidelines remain to be established. Rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (R-EPOCH) has been proposed, given that it involves a continuous infusion of anthracycline, reducing the risk of a cardiotoxicity and therefore the theoretical risk of perforation. However, the literature on this method of treatment is scarce. Herein, we present a unique case of a 75-year-old male, diagnosed with primary cardiac diffuse large B-cell lymphoma (DLBCL) with relatively unusual involvement of the coronary sinus, treated first with one cycle of R-EPOCH, followed by three cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) to reduce said risk. To our knowledge, this is one of two cases, in which a patient with PCL was treated this way.
... Morphologically it resembles a sea anemone with frond-like arms and a stalk. Histologically they consist of endothelial cells surrounding the connective tissue core [3]. ...
... Papillary fibroelastomas (PFE) represent a rare but noteworthy subset of benign cardiac tumors, predominantly afflicting the valvular endocardium, particularly favoring the left-sided valves. Despite their infrequency, they hold significance in clinical practice, constituting approximately 11.5% of all primary cardiac tumors and often manifesting with embolic complications, particularly prevalent among middleaged individuals [1]. These tumors exhibit a characteristic slow growth, with an average annual rate of 0.5 ± 0.9 mm/year, and display a diverse range in size, spanning from 2 mm to 70 mm, with a mean diameter of 9 mm [2]. ...
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Cardio-oncology, at the intersection of cardiovascular diseases, oncological conditions, and treatments, presents unique challenges in medical care. This abstract highlights a case involving a 60-year-old male presenting with syncope at work; the workup revealed a rapidly growing tricuspid valve papillary fibroelastoma (PFE), emphasizing diagnostic approaches, management strategies, and clinical implications. The diagnostic investigation, including blood cultures, transthoracic echocardiogram, transesophageal echocardiogram, and cardiac MRI, confirmed the diagnosis of tricuspid valve PFE. A multidisciplinary approach led to a shared decision with the patient to opt for serial monitoring. Syncope was attributed to dehydration. This case underscores the complexities of managing cardiovascular conditions in the context of oncology and the importance of collaborative decision-making in patient care.
... Primary tumors of the pericardium can present with constrictive physiology. The neoplasm encasing the heart can be revealed by TTE, along with the pericardial effusion [49]. The imaging features on CT and CMR overlap with inflammatory pericarditis and include diffuse pericardial thickening and nodular enhancement. ...
... It must be stressed that pericardiocentesis yields positive cytology in less than 50% of cancer-related pericardial effusions [57][58][59]. A frankly hemorrhagic pericardial fluid suggests that pericardial effusion is secondary to cancer metastases or primary angiosarcoma [9,49]. Histopathologic evaluation of pericardial biopsy specimens collected by means of thoracoscopy or thoracotomy is another option to determine the etiology of pericardial effusion, but it is seldom followed [56]. ...
... Pericardial effusion in the setting of post-actinic acute pericarditis is generally benign and does not impose to stop cancer therapy [49]. ...
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Background This article provides an up-to-date overview of pericardial effusion in oncological practice and a guidance on its management. Furthermore, it addresses the question of when malignancy should be suspected in case of newly diagnosed pericardial effusion. Main body Cancer-related pericardial effusion is commonly the result of localization of lung and breast cancer, melanoma, or lymphoma to the pericardium via direct invasion, lymphatic dissemination, or hematogenous spread. Several cancer therapies may also cause pericardial effusion, most often during or shortly after administration. Pericardial effusion following radiation therapy may instead develop after years. Other diseases, such as infections, and, rarely, primary tumors of the pericardium complete the spectrum of the possible etiologies of pericardial effusion in oncological patients. The diagnosis of cancer-related pericardial effusion is usually incidental, but cancer accounts for approximately one third of all cardiac tamponades. Drainage, which is mainly attained by pericardiocentesis, is needed when cancer or cancer treatment-related pericardial effusion leads to hemodynamic impairment. Placement of a pericardial catheter for 2-5 days is advised after pericardial fluid removal. In contrast, even a large pericardial effusion should be conservatively managed when the patient is stable, although the best frequency and timing of monitoring by echocardiography in this context are yet to be established. Pericardial effusion secondary to immune checkpoint inhibitors typically responds to corticosteroid therapy. Pericardiocentesis may also be considered to confirm the presence of neoplastic cells in the pericardial fluid, but the yield of cytological examination is low. In case of newly found pericardial effusion in individuals without active cancer and/or recent cancer treatment, a history of malignancy, unremitting or recurrent course, large effusion or presentation with cardiac tamponade, incomplete response to empirical therapy with nonsteroidal anti-inflammatory, and hemorrhagic fluid at pericardiocentesis suggest a neoplastic etiology.
... 3,6 These cells are spatially closely associated with vascular ECs and typically form myxomatous perivascular ring structures (single or multilayered) or pseudo-vascular structures. 33 These observations have led to the impression that vascular channels seem to arise from the myxomatous structures. 6,34 However, due to technical limitations, there is no evidence supporting the view that the ECs of the vascular channel in CM could be directly derived from myxoma tumour cells. ...
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Background Cardiac myxoma (CM) is the most common (58%–80%) type of primary cardiac tumours. Currently, there is a need to develop medical therapies, especially for patients not physically suitable for surgeries. However, the mechanisms that shape the tumour microenvironment (TME) in CM remain largely unknown, which impedes the development of targeted therapies. Here, we aimed to dissect the TME in CM at single‐cell and spatial resolution. Methods We performed single‐cell transcriptomic sequencing and Visium CytAssist spatial transcriptomic (ST) assays on tumour samples from patients with CM. A comprehensive analysis was performed, including unsupervised clustering, RNA velocity, clonal substructure inference of tumour cells and cell–cell communication. Results Unsupervised clustering of 34 759 cells identified 12 clusters, which were assigned to endothelial cells (ECs), mesenchymal stroma cells (MSCs), and tumour‐infiltrating immune cells. Myxoma tumour cells were found to encompass two closely related phenotypic states, namely, EC‐like tumour cells (ETCs) and MSC‐like tumour cells (MTCs). According to RNA velocity, our findings suggest that ETCs may be directly differentiated from MTCs. The immune microenvironment of CM was found to contain multiple factors that promote immune suppression and evasion, underscoring the potential of using immunotherapies as a treatment option. Hyperactive signals sent primarily by tumour cells were identified, such as MDK, HGF, chemerin, and GDF15 signalling. Finally, the ST assay uncovered spatial features of the subclusters, proximal cell–cell communication, and clonal evolution of myxoma tumour cells. Conclusions Our study presents the first comprehensive characterisation of the TME in CM at both single‐cell and spatial resolution. Our study provides novel insight into the differentiation of myxoma tumour cells and advance our understanding of the TME in CM. Given the rarity of cardiac tumours, our study provides invaluable datasets and promotes the development of medical therapies for CM.
... Embryonal rhabdomyosarcoma is a characteristically aggressive malignant tumor with up to 46.0% of metastases found at diagnosis. The brain, lung, liver, and bone are the most common sites of dissemination [5][6][7] . In our case report, no invasion of adjacent structures or metastases was found despite its large size. ...
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Cardiac rhabdomyosarcomas are rare in adults and are symptomatic when they become large or invade adjacent structures. We present the case of a 30-year-old woman with a left atrial tumor detected with contrast-enhanced computed tomography (CECT) and transthoracic echocardiogram (TTE). The patient presented severe mitral stenosis with heart and respiratory failure, requiring advanced airway management and emergency cardiac surgery. CECT showed a filling defect in the left atrium caused by a 66 x 37 x 33 mm, oval, hypodense, and homogeneous solid mass with a well-defined margin occupying the left atrium. The mass originated from the atrial septum and invaded the right atrium. Mild enhancement was noted in the contrast phase. TTE showed a mobile hyperechogenic, homogeneous, non-vascularized mass with a well-defined margin adhering to the interatrial septum and prolapsing into the left ventricle during atrial systole, causing severe mitral stenosis. The tumor was excised, and a diagnosis of embryonal rhabdomyosarcoma was confirmed. This case is the first reported in Mexico of a cardiac embryonal rhabdomyosarcoma detected in the left atrium in an adult. CECT and TTE imaging findings are reported for educational purposes. Keywords: Cardiac tumors. Embryonal rhabdomyosarcoma. Rhabdomyosarcoma. Primary cardiac tumors. Case report.
... Image Credit: Tyabelly et al., 2020 [33]; Licensed under CC-BY ...
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While primary cardiac malignancies are infrequent, the heart often serves as a site for metastases. Myxomas are recognized as among the most prevalent primary benign tumours globally, while sarcomas represent the most common malignant primary tumours. The diverse range of potential clinical presentations depends on factors such as location, size, and the aggressiveness of the disease. The majority of diagnoses rely on medical imaging, making it crucial to familiarize oneself with their distinctive characteristics. When a cardiac mass is suspected, MRI of the heart has emerged as the preferred diagnostic method, surpassing previous techniques. CT is a valuable tool for assessing cardiac morphology and improving electrocardiography gating by providing enhanced details. This article conducts a comprehensive review of the MRI and CT characteristics of both primary and secondary cardiac malignancies, emphasizing crucial distinctions and common diagnostic pitfalls. Despite their rarity, cardiac masses continue to hold significance in the realm of cardio-oncology. Furthermore, this article explores conditions such as thrombus, Lambl's excrescences, and pericardial cysts, which can mimic tumours. Multimodal imaging has played a pivotal role in identifying the origin of cardiac masses in numerous cases, particularly when combined with the clinical context. This article offers an in-depth examination of the frequency, clinical indicators, imaging, diagnostic procedures, available treatments, and prognoses related to cardiac masses.