Oncotic (Myxomatous) Aneurysms: A Review of Management

Atrial myxomas are the most common primary cardiac tumors and may manifest with neurological symptoms in ∼ 30% of cases. Cerebral ischemia, aneurysmal formation, and extravascular metastases are mechanisms that lead to these neurological manifestations. Perilesional changes on computed tomography (CT) and magnetic resonance imaging (MRI) may help in the diagnosis of myxomatous aneurysms, which are usually located in the distal middle cerebral artery (MCA) and in the posterior cerebral artery (PCA) circulation territories. Careful resection of the cardiac lesion is essential for preventing embolism. However, treatment of myxomatous aneurysms is controversial due to the limited understanding of the natural history of this condition. Treatment may include clinical observation in asymptomatic patients, surgical resection, endovascular approaches, adjuvant chemotherapy, and low-dose radiation therapy. We present one case of a female patient with myxomatous aneurysm secondary to an atrial myxoma who presented with neurological symptoms and another case of a female patient who developed neurological symptoms after initial surgical treatment of the primary lesion. Lesion growth rate, topography, morphology, and the patient's clinical condition must be considered when choosing a therapeutical method. Further clinical studies are needed to achieve a better understanding and treatment of this disease.

261 A case of metastatic myxoid liposarcoma causing cardiac tamponade

Aims Primary cardiac tumors are generally benign. In one series of over 12 000 autopsies, only seven cases of malignant primary cardiac tumors were identified, for an incidence of less than 0.1%. By comparison, metastatic involvement of the heart is over 20 times more common and has been reported in autopsy series in up to one in five patients dying of cancer. Myxoid liposarcoma (MLS) is the second most common subtype of liposarcoma and it usually occurs in deep tissues of the extremity, especially in the calf or thigh. Some tumors have round cell areas that represent histologic progression to high-grade tumours. Round cells, defined as > 5% of the total cells, are associated with higher malignancy and metastatic potential, resulting in an unfavourable outcome in patients affected. Patients with MLS tend to have metastases to extra pulmonary sites, such as abdominal wall, abdominal cavity, retroperitoneum, and bone, even in the absence of pulmonary metastases. Although several authors have reported a high proportion of extra pulmonary metastases of MLS, cardiac metastasis is extremely rare. Methods and results We present a case of a young woman who underwent resection of calf liposarcoma with the onset of cardiac metastases after 18 years, with cardiac tamponade as a clinical onset. MRI confirmed the cardiac solid mass already evident on CT scan, located along the free anterior wall of the right ventricle in the mid-basal area; the lesion seemed to have pericardial implantation with the free wall of the right ventricle was not well cleaved by the lesion in the Cine-MRI sequences. However it was always visible in the post-contrast sequences without evident protrusion of the lesion into the cavity or signs of thrombosis. The parietal pericardium was located on the periphery of the lesion and was not recognizable in some points. The lesion consisted of two portions, a lower one in which some components with characteristics of adipose signal were recognizable, while the upper one was more solid and vascularized. There was persistence of inhomogeneous and diffuse late enhancement at the lesion level. PET revealed pathological hyper accumulations of radiopharmaceuticals in the heart lesion along the anterior wall of the right ventricle, with central hypocaptation area, possible expression of necrotic-colliquative phenomena. Other hyper accumulations were found in the mediastinal lymph node in the pre-carenal area. These findings were referable to the presence of glucose hyper metabolic tissue of suspected neoplastic significance. The patient temporarily declined surgical excision, but after one month, due to the worsening of her symptoms, she returned to emergency room: The CT scan showed a volumetric increase in cardiac mass with a vertical diameter of 8 cm and adhering to the right ventricle for 7 cm. Thanks to the possibility of surgery, patient underwent exeresis of the capsulated epicardial mass adhering to the anterior wall of the right ventricle and to the pericardium, with removal of the ventricular wall and reconstruction with a bovine pericardium patch and without postoperative complications. Conclusions In MLS the time intervals between the onset of primary disease and cardiac metastasis were reported to be relatively long, ranging from 1 to 25 years. The initial site of metastatic disease in our patient was the heart: she had a solitary cardiac tumour that presented 18 years after the primary surgery in the absence of metastases at other sites. When a cardiac metastasis is found incidentally, it is usually incurable.

Abstract 17074: Primary Cardiac Sarcoma In Elderly Patient And Need For High Index Of Suspicion

Case Presentation: Primary cardiac tumors are rare with an incidence between 0.0017 and 0.2%. Most cardiac tumors are benign such as myxomas, lipomas, and papillary fibroelastomas. Primary malignant cardiac tumors are even more uncommon, with cardiac sarcomas being the most common. We present a case of an 87-year old female who presented initially for progressive shortness of breath and concern for potential endocarditis vs intra-cardiac thrombus noted on outside hospital transthoracic echocardiogram. Blood cultures were negative, no leukocytosis or sequelae of infection were noted. Transesophageal echocardiogram (TEE) noted a large lesion attached to the anterior mitral leaflet, with additional lesions noted on the left atrial free wall just medial to the right sided pulmonary veins. Obstruction of mitral inflow was noted with a mean gradient through the mitral valve of 5mmHg. Cardiac MRI was performed for further delineation of lesions and based on tissue characteristics were felt to be thrombus (Figure 1). Hypercoagulability workup was negative, and there was no improvement in lesion size with systemic anticoagulation. Cardiothoracic surgery was consulted for surgical removal of the mass. Pre-operative cardiac catheterization showed multi-vessel obstructive coronary artery disease. It was also noted that the left circumflex artery appeared to supply blood into a discrete area in the left atrium. The patient was ultimately taken for surgical removal. Surgical pathology demonstrated primary high grade cardiac sarcoma. Discussion: The rarity and heterogeneity of primary cardiac tumors, along with many potential mimickers pose a diagnostic and therapeutic challenge. This, combined with the location and aggressive nature of the tumors, make them difficult to treat and incur a high mortality rate. This highlights the importance of maintaining a high index of suspicion and keeping a broad differential when evaluating intra-cardiac masses.

Rare Case of 6 cm Right Atrial Myxoma in Patient with Synchronous Endometrial Adenocarcinoma

Primary cardiac tumors are extremely rare. Cardiac myxomas most frequently appear in the left atrium. In this article, we present a case of an asymptomatic 6 cm right atrial mass in a patient undergoing staging for endometrial cancer. The mass was resected, and final pathology was consistent with cardiac myxoma.

PRIMARY CARDIAC TUMORS: EARLY SURGERY, COMPLETE RESECTION, CHEMOTHERAPY ENHANCES SURVIVAL.

Primary cardiac tumors are rare. The incidence varies between 0.3% to 0.7% .Quarter of all primary cardiac tumors are malignant, of these 75% are sarcomas. Malignant primary cardiac sarcomas that affect right atrium are predominantly angiosarcomas while the left atrium is involved primary in pleomorphic sarcoma like malignant brous histiocytoma and leiomyosarcoma. In a young patient it usually carries a dismal prognosis if not diagnosed early and dealt with surgically followed by adjuvant therapy. Without surgical resection, the survival rate at 1yr is only 10%.Retrospective evaluation of the last 60 primary cardiac tumors done by the same surgical team over 28 years was looked into.

Clinical Features, Echocardiographic Findings, Management and Outcome in a Young Male Patient with Atrial Myxoma: A Rare Primary Cardiac Tumor

Primary cardiac tumors in pediatric are rare and mostly benign. Atrial Myxoma is the most common primary tumor of the heart, which is usually involved in the left atrium. Surgical treatment is recommended when the patients develop symptoms or hemodynamically is impaired. Cardiac Myxoma can cause left atrial obstruction and systemic embolization. So early diagnosis with surgical intervention is the definitive management of the tumor and should not be delayed. Here we present a case of a 23-year old male who admitted to the emergency department with syncope and dyspnea for 1day ago. The tumor was removed surgically; pathologic examination confirmed the diagnosis of Myxoma.

Ultrasonic fi nding of dumbbellshaped lipomatous hypertrophy of interatrial septum

Benign lipomatous formations rank second in frequency among primary cardiac tumors. According to the degree of encapsulation, they are divided into two groups — adipose tumors and lipomatous hypertrophies of interatrial septum. Cases of lipomatosis of the heart and lipomatous hypertrophy of interatrial septum were described in scientifi c editions in years past. Interest in this pathology is related to the question: is it necessary to perform preventive surgical intervention before the appearance of clinical signs of endocardial hemodynamics disturbance? Moreover, the risk associated with surgery is much higher than the existence of a benign hyperplastic process in the anatomical structures of the heart.