A case report of a malignant melanoma in the cardiologic diagnostic workup.

Background: Cardiovascular imaging plays an important role in identifying pre-existing cardiac comorbidity prior to the decision on cancer therapy and serves as a reference for detecting changes during treatment and long-term follow-up and also in the further identification of a possible cardiac manifestation of the underlying oncological disease. Case summary: We report the case of an 81-year-old patient with a malignant melanoma. The patient initially was presented before the start of adjuvant therapy with serine/threonine-protein kinase B-Raf/mitogen-activated extracellular signal-regulated kinase inhibitors. Cardiologic staged diagnostics using transthoracic echocardiography, transoesophageal echocardiography, and cardiovascular magnetic resonance imaging (CMR) revealed with a high probability a cardiac manifestation of the underlying disease. The echocardiographic and CMR results as well as the diagnostic workup are presented. Discussion: Cardiac masses in general have a variety of differential diagnoses. Cardiac metastases are much more common than primary neoplasms in a ratio of about 10:1. Cardiovascular risk stratification is recommended in all patients with cancer before starting potentially cardiotoxic anticancer therapy. Cardiovascular imaging plays an important role for baseline risk stratification but is also the leading diagnostic tool in the differential diagnosis of cardiac tumours and the planning of a potential therapy.

Incidental Finding of Atrial Myxoma in a Patient Presenting With Transient Ischemic Attack (TIA): A Case Report.

Atrial myxomas are benign primary cardiac tumors. They can present with nonspecific symptoms, ranging from constitutional symptoms and embolic phenomena such as transient ischemic attacks (TIAs) or strokes to sudden cardiac death. Early diagnosis may be a challenge due to the nonspecific presentation of atrial myxoma. A high degree of suspicion is needed in patients with TIA having no known cardiovascular risk factors. Although benign, if left untreated, it can lead to serious complications ranging from embolic phenomena and obstructive symptoms to sudden cardiac death. An echocardiogram is of fundamental importance in diagnosing atrial myxoma, and surgical resection is the ultimate treatment of choice. Here, we discuss a case of TIA as the initial presentation of atrial myxoma.

Successful surgical resection of a metastatic clear cell sarcoma in the heart: a case report.

Background: Clear cell sarcoma (CCS) is a very rare disease and one with a very poor prognosis. Furthermore, its occurrence in the heart is very rare and past reports are scarce. Case summary: A 33-year-old man who had undergone left arm amputation due to CCS came to the hospital because a positron emission tomography computed tomography (PET-CT) four years post-amputation showed an accumulation in the heart. The PET-CT with glucose suppression treatment showed fluorodeoxyglucose accumulation in the myocardium between the middle of the anterolateral wall and the papillary muscle of the posterior lateral wall of the left ventricle (LV). Based on the course of the disease up to now, it was considered that the accumulation was most likely metastasis of CCS. Observation of the heart after a median sternotomy revealed a white tone, well-defined lesion in the middle of the anterolateral wall of LV. The tumour on the posterolateral side of LV was not exposed on the surface, but it was palpated and was still recognizable as a firm neoplastic lesion. Because the mass was identified as a sarcoma on intraoperative rapid pathology, we decide to perform a total resection. Both lesions were excised, and pathology revealed a diagnosis of CCS. Discussion: Clear cell sarcoma is a very rare disease that accounts for <1% of all soft tissue sarcomas, and its occurrence in the heart is even rarer. It requires a combination of many imaging modalities. To our knowledge, this is the first case of CCS in the heart treated with surgical resection.

[You only ECHO Twice].

INTRODUCTION: Ischaemic stroke results from a sudden interruption of cerebral blood flow, often caused by thrombosis, embolism or hypoperfusion. Rarely, cardiac tumours are the cause of an embolic event. We report on an 80-year-old woman with a cerebral stroke. Successful thrombectomy revealed a histopathologically confirmed papillary fibroelastoma as the embolic source. Although it was not detectable on initial echocardiography, the tumour was discovered by transesophageal echocardiography. This case emphasises the importance of considering primary cardiac tumours as potential sources of emboli in ischaemic stroke. Keywords.

Primary left ventricular leiomyosarcoma: a case report.

Cardiac leiomyosarcomas are a rare subset of the already infrequent, primary malignant cardiac neoplasia spectrum. The most common site for a primary leiomyosarcoma of the ventricle is on the right with fewer than five globally reported cases in the left ventricle. Most present with non-specific symptoms but attention is usually sought after the appearance of compressive symptoms or arrhythmias. We present a case of a left ventricular leiomyosarcoma in a 50-year old female patient that had a delayed diagnosis and its subsequent surgical resection and oncological management with docetaxel and gemcitabine. This case highlights the need for a high index of suspicion for cardiac masses especially if there are competing chronic diseases with similar symptomatology. Given the rare presentation of left ventricular leiomyosarcomas, case reports may provide valuable information that is otherwise unavailable.

Epicardial cavernous haemangioma; A case report of a unique incidental finding.

Background: Primary cardiac tumours are rare, accounting for only 0.002-0.03% at autopsy. Cardiac haemangiomas are benign vascular tumours and constitute for 0.28% of all primary cardiac tumours. Cavernous haemangiomas, capillary haemangiomas, and arteriovenous haemangiomas are three distinct types. Cardiac haemangiomas are often misdiagnosed as myxomas and must be differentiated from malignant angiosarcomas. Case summary: We present a 44-year-old Mediterranean male patient with a cavernous haemangioma in the inferior vena cava and right atrium, detected on transthoracic echocardiography. The patient experienced palpitations and dyspnoea on exertion. Computed tomography (CT) angiography revealed a 7.5 × 6 × 5 cm mass suspected to be perfused by the distal right coronary artery. A watch-and-wait approach was suggested, leading to a cardiac magnetic resonance imaging (MRI) with contrast 6 months later. T1 mapping exhibited a prolonged relaxation time and isointensity to the myocardium. T2 mapping revealed a homogenous hyperintense mass with heterogenous late enhancement. Surgical excision was performed using a bicaval cannulation technique on cardiopulmonary bypass. Intraoperatively, no connection to the coronaries was noted. At 1 year follow-up, the patient reported restored physical resilience, with no evidence of tumour recurrence. Discussion: Clinical symptoms of cardiac cavernous haemangiomas are unspecific and become evident once the tumour grows. To investigate the nature and vascular involvement of the tumour, a contrast-enhanced CT angiography or MRI can be performed. Cardiac haemangiomas are often misdiagnosed and must be differentiated from malignant angiosarcomas. Clear guidelines for the treatment of cardiac haemangiomas in adult patients are lacking. Primary cardiac tumours require thorough investigation, and surgical intervention should be tailored to the individual's case.

Cardiac paraganglioma: implications and impacts of a rare disease-a case report †.

Background: Tumour-producing catecholamines arise in the adrenal medulla (pheochromocytomas), as well as in extra-adrenal chromaffin cells (paragangliomas). The origin can be from any location; however, it is very rare in the heart. Case summary: A 43-year-old woman with a history of arterial hypertension presented with dyspnoea on moderate exertion, New York Class Association (NYHA) functional classes III and IV, and oedema in the lower extremities. Medical and laboratory evaluation revealed an NT-proBNP of 6046 pg/mL, a left ventricular ejection fraction (LVEF) of 15%, longitudinal strain of -7%, and a mass located on the inner surface of the left atrioventricular groove. Surgical intervention was performed, and the tumour was resected. Pathological report showed an extra-adrenal paraganglioma without neoplastic involvement in the margins of the vena cava. After surgery, the patient showed clinical improvement with NYHA functional class I, LVEF of 56%, and longitudinal strain of -20% on transthoracic echocardiography 4 months after treatment. Discussion: Paragangliomas are tumours that are rarely found in the heart, and their diagnosis is difficult. However, early detection and treatment can improve the quality of life of affected patients.

Rare cardiac tumour-nodular fasciitis.

Left ventricular tumour is a rare condition in children. The causes include vegetations, thrombus, and fibroma. 2-year-old asymptomatic female presented with an innocent heart murmur at 6 months of age. Subsequent follow-ups at 18 months of age showed left ventricular mass. Surgical pathology revealed "nodular fasciitis." This type of tumour has never been described in the heart before.

Hibernoma: a case report of a rare cardiac tumour.

Background: A cardiac hibernoma is a rare phenomenon, with just a handful of reports in the literature. They are difficult to characterize with conventional imaging including echocardiography, computed tomography (CT), cardiac magnetic resonance (CMR), or positron emission tomography (PET). Their definitive diagnosis relies primarily on histopathology via either endovascular or surgical biopsy. Previous case reports have entailed surgical excision followed by histopathology; however, surgery may be unfavourable in some patients with increased perioperative risk. Case summary: We present the case of a 57-year-old woman who was referred to our cardiology service with an interatrial lipomatous mass found incidentally on chest CT for assessment of rib fractures. She had 6 months of unexplained syncope, which was attributed to superior vena cava (SVC) compression demonstrated by chest CT. The mass had benign characteristics on echocardiography, CT, and CMR but was glucose-avid on PET, which indicated a possible malignancy such as liposarcoma. Her comorbid and very significant airways disease precluded her from surgical excision, so instead, endovascular biopsy was performed. Histopathology showed brown fat which was negative for mouse double minute 2 amplification on fluorescence in situ hybridisation testing; hence, a diagnosis was made of hibernoma, a rare benign tumour of brown fat. Given the benign diagnosis and her surgical risk with severe chronic obstructive pulmonary disease, a multidisciplinary recommendation was made favouring conservative management, with careful ongoing follow-up and the consideration of SVC stenting if symptoms progressed. Discussion: The definitive diagnosis of a cardiac hibernoma is complex and relies heavily on histopathology due to the contradictory findings on chest imaging. Careful consideration of management within a multidisciplinary team setting is essential to achieve a successful outcome.

Left-sided primary cardiac lymphoma diagnosed by needle biopsy with total endoscopic anterolateral mini-thoracotomy: a case report.

Background: Primary cardiac lymphoma (PCL) is an extremely rare tumour that typically affects the right heart chamber. It is a life-threatening tumour presenting with rapid growth; therefore, early diagnosis and treatment are crucial for improving the prognosis of patients with PCL. Case summary: An 81-year-old female with a history of dermatomyositis and interstitial pneumonia was referred to the cardiology department for cardiomegaly detected on chest radiography and computed tomography (CT). She experienced shortness of breath on exertion. Electrocardiography revealed negative T-waves in various leads. Transthoracic and transoesophageal echocardiography revealed a large mass on the epicardial free wall of the left atrium and ventricle. Coronary CT angiography showed feeding vessels from the left circumflex artery and the posterolateral branch of the right coronary artery. Positron emission tomography showed elevated mass uptake and no systemic metastasis. Needle biopsy with total endoscopic anterolateral mini-thoracotomy was performed. Histopathological examination revealed diffuse large B-cell lymphoma. She received systemic chemotherapy and achieved a complete metabolic response. Discussion: Herein, we report an extremely rare case of PCL located on the left side of the heart. Owing to the location of the tumour, percutaneous or transcatheter biopsy could not be performed. Early diagnosis with needle biopsy via anterolateral mini-thoracotomy and systemic chemotherapy resulted in good outcomes.

Case series: cardiac sarcoma.

Background: Cardiac masses encompass a wide differential including primary and secondary malignancies and can present with a variety of symptoms, many of which are non-specific. Early identification and classification are important, particularly for cardiac malignancies such as sarcomas as these are aggressive tumours with exceptionally poor prognoses when metastases are present at diagnosis. Case summary: We report two cases of patients who presented with dyspnoea and were diagnosed with cardiac sarcomas; the former a primary sarcoma (undifferentiated pleomorphic subtype) and the latter a secondary sarcoma (round cell myxoid liposarcoma) that serve as comparisons for presentation and management of different types of this disease. Computed Tomography (CT) and echocardiography imaging findings are demonstrated showing the typical location and morphology of each subtype. Discussion: Cardiac sarcomas are the most common primary cardiac malignancy, of which undifferentiated pleomorphic sarcoma is a common subtype. Undifferentiated pleomorphic sarcomas are aggressive, have a tendency to arise in the left atrium, and can appear similar to benign cardiac masses. Round cell myxoid liposarcomas by contrast are rare causes of secondary cardiac malignancies, metastasizing to the heart from soft tissues. Both diagnoses carry poor prognoses and although rare, are important to recognize as timely intervention with surgery, radiotherapy, and consideration of chemotherapy is key to maximizing survival.

The role of single-cell RNA sequencing in cardiac tumour - a case report.

A 65-year-old woman presented to our hospital with 5 days of chest tightness, dyspnoea, and lower abdominal distension. Echocardiography revealed a mass in the right atrium. An emergency operation was carried out to prevent tumour shedding. The patient was discharged on the 4th day of tumour resection, without any complications At the 18 months follow-up, she suffered from kidney and lung tumours. She refused any treatment and passed away. scRNA-seq was applied to analyse the nature of the tumour. The cellular components of benign tumours include chondrocytes, smooth muscle cells, fibroblasts, mesenchymal stromal cells, and osteoblasts. Additionally, the cyclic guanosine monophosphate (cGMP-PKG) signalling pathway, transcriptional misregulation in cancer, and the p53 signalling pathway may be related to the growth of this tumour. scRNA-seq is a good approach to analyse growth patterns of cardiac tumours and helpful for distinguishing the nature of the tumour.

Pulmonary valve spindle-cell lipoma: A case report.

Primary cardiac tumours are rare and most of them are benign. Myxomas, fibroelastomas and lipomas are common in adults. Primary valvular cardiac tumours are even more rare and affect all four valves in a similar proportion. Valvular lipomas are very rare. In the pulmonary valve there is only one described. Lipomas can be spindle-cell varieties. But of these, there is only one described in a valve, and it is placed in the aortic valve. Pulmonary valve lipomas can produce obstruction to the right ventricular outflow tract as well as pulmonary valve regurgitation, or pulmonary embolism. Symptoms may be dyspnoea, angina, arrhythmias, or syncope. We aim to illustrate with this case report how we came into this very rare pathology, so we present a 54-year-old woman with a giant spindle-cell lipoma located in the anterior pulmonary leaflet and severe dyspnoea. Total resection of the tumour was performed and restoration of valve function was obtained by means of bicuspidization of the remaining pulmonary leaflets. She had a good recovery after surgery and no complication during the postoperative evolution, being discharged from hospital after 7 days from surgery, with echocardiographic control showing good biventricular function, absence of tumour or obstruction, and minimal pulmonary valve regurgitation.

Brugada phenocopy with altered ST-segment elevation in pericardial diffuse large B-cell lymphoma and effusive-constrictive pericarditis: a case report.

Background: Cardiac lymphoma is a rare disease. Effusive-constrictive pericarditis can be a characteristic of pericardial involvement in patients with this disease. Conversely, a phenotype with electrocardiogram changes similar to those of Brugada syndrome is called Brugada phenocopy, and these changes improve after treatment. Case summary: A 71-year-old man was transported to our hospital with chest pain, hypotension, and ST-segment elevation in V1 and V2 leads during maintenance dialysis for renal failure. After arrival at the hospital, his ST-segment elevation disappeared, and emergency coronary angiography scan revealed no significant coronary artery stenoses or obstructions. His computed tomography and echocardiography scans revealed pericardial effusion and an intrapericardial mass. Further, his blood pressure dropped and ST-segment elevation recurred during dialysis after 7 days. Thus, pericardiocentesis was performed, but haemodynamic improvement was insufficient, and right catheterization findings suggested effusive-constrictive pericarditis. Meanwhile, flow cytometry of the pericardial fluid suggested the diagnosis of B-cell lymphoma; however, radical chemoradiotherapy was impossible because of cardiogenic shock. The patient died on Day 17. Further, autopsy revealed diffuse large B-cell lymphoma with pericardial and myocardial infiltration. Discussion: Cardiac lymphoma is rare but can be associated with effusive-constrictive pericarditis, which may be difficult to manage even with pericardial drainage. In such cases, radical treatment, including chemotherapy, should be promptly considered, if possible. Our patient presented with Brugada-type electrocardiogram but no syncope or family history, suggesting Brugada phenocopy and not true Brugada syndrome due to cardiac lymphoma. Notably, temporary improvement in ST-segment elevation was observed despite the absence of treatment.

Pulmonary valve papillary fibroelastoma: to PE or not to PE.

Pulmonary valve (PV) fibroelastomas are a rare pathology, with limited anecdotal literature surrounding them. Consequently, the natural history is unclear; however, two features have remained salient; they are asymptomatic and found incidentally. Here, we describe a 52-year-old female, presenting with symptoms suggestive pulmonary embolism (PE). Pulmonary angiography revealed a filling deficit in the pulmonary trunk (PT), adjacent to the PV. Subsequent investigation found a large PV fibroelastoma. The presence of symptoms is likely secondary to right ventricular outflow tract obstruction from the lesions large size. We describe our investigation and management of the lesion. The reporting of this case challenges the existing knowledge of PV fibroelastomas.

A rare case of cardiac myxoma with light bulb-like cystic morphology: a case report.

Background: Cystic myxomas are quite rare. Moreover, few reports have evaluated the causes that constituted them. Case summary: A 73-year-old Asian man presented for pre-operative examination of osteoarthritis, and transthoracic echocardiography (TTE) revealed an incidental intracardiac mass. Therefore, he was referred to our department for further evaluation. He had no specific symptoms or family history related to tumours and heart failure. The TTE showed a 32 × 24 mm spherical mass adherent to the left atrial septum. The upper part of the mass was cystic in formation and hypoechoic inside and resembled a light bulb. Transoesophageal echocardiography showed the feeding arteries flowing from the bottom into the cystic part. In addition, two jet strips drained from the cystic part in the direction of the mitral valve. Coronary angiography revealed the feeding arteries, which consisted mainly of the right coronary artery conus branch and the left circumflex branch, and the blood flowed into the saccular area from the feeding arteries and excreted towards the mitral valve. Surgical resection was performed due to the mobility, and the histopathology confirmed a cystic myxoma. Discussion: We described the unique anatomical formation of a cystic myxoma, which consisted of an exquisite balance between the tumour-feeding arteries and the draining outlet vessels.