1/49. leiomyosarcoma of the heart and its pulmonary metastasis, both with prominent osteoclast-like multinucleated giant cells expressing tartrate-resistant acid phosphatase activity.An autopsy case of cardiac leiomyosarcoma and its pulmonary metastasis, both with osteoclast-like multinucleated giant cells (OMGC) mimicking the so-called giant cell variant of malignant fibrous histiocytoma (MFH), is reported. The patient, a 70-year-old male, was admitted for sudden dyspnea. Extensive work-up established only a left atrial tumor mass. Three months after admission, the patient developed multiple intracranial and pulmonary metastases, followed by a worsening clinical course characterized by semicoma and dyspnea, and subsequently died 6 months after the onset of his symptoms. At subsequent autopsy, the left atrial polypoid tumor was found to have invaded destructively to the left half of the cardiac wall. histology of the cardiac tumor revealed a bimorphic sarcoma in which a poorly differentiated leiomyosarcoma comfirmed by histologic and immunohistochemical findings was juxtaposed to a small nodule with features closely mimicking giant cell MFH. The pulmonary metastatic nodules exhibited features that were entirely indistinguishable from giant cell MFH except for the fact that a minority of polymorphic cells manifested myogenic differentiation. We believe that such a MFH-like pattern represents a pleomorphic form of leiomyosarcoma rather than a dedifferentiated one. The OMGC within the MFH-like component coexpressed CD68 and tartrate-resistant acid phosphatase activity.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/49. Treatment of life-threatening huge atrial myxoma: report of two cases.We herein report two patients with left atrial myxoma who needed an emergency operation. Case 1 was a 48-year-old woman who was injured in a traffic accident and underwent an operation for a right leg fracture. Just after the operation she developed cardiac and respiratory arrest with complaints of chest pain. She was successfully resuscitated and diagnosed to have a left atrial myxoma by echocardiography. Emergency surgery was performed and a giant left atrial myxoma was thus removed from the atrial septum. Case 2 was a 54-year-old housewife who was transferred to our department under the diagnosis of a left atrial myxoma by echocardiography. She complained of dyspnea and chest discomfort. By angiography, the tumor was seen to be partially incarcerated at the diastolic phase. A huge myxoma was removed from the atrial septum which was secured by a patch closure. Cardiac echocardiography can help rule out left atrial myxoma if it is highly suspected. As early surgical mortality is low and the long-term results are good, we strongly believe that patients with cardiac myxoma should be operated on as early as possible, once a diagnosis is made.- - - - - - - - - - ranking = 0.125keywords = giant (Clic here for more details about this article) |
3/49. A survivor of near sudden death caused by giant left atrial myxoma.Sudden hemodynamic collapse occurred in a 20-year-old man after an Emergency Department visit with a complaint of dizziness and chest discomfort. A left atrial myxoma was demonstrated by echocardiography. resuscitation procedures followed by surgical repair resulted in an excellent outcome. Although sudden death is a serious manifestation of cardiac myxoma, reports of survivors of near sudden death caused by this tumor have been rare.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
4/49. A large false aneurysm of the right ventricle within a giant epicardial lipoma.Lipomas, which account for approximately 10% of all neoplasms of the heart, may be detected in asymptomatic patients by chance during echocardiography, CT scan, or MRI scan. Occasionally, lipomas are complicated by arrhythmias. We describe a patient who presented with severe cardiomegaly and paroxysmal supraventricular tachycardia. An MRI scan showed a large intrapericardial lipoma with two large cavities inside communicating with each other and with the right ventricular chamber through a defect of the right ventricular wall. The mass was partially removed, and the right ventricle was patched. Surgery combined with antiarrhythmic therapy resulted in a good short-term result.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
5/49. A t(2;19)(p13;p13.2) in a giant invasive cardiac lipoma from a patient with multiple lipomatosis.Cardiac lipomas occur infrequently but account for a significant portion of rare cardiac tumors. Common cutaneous lipomas have previously been associated with rearrangements of chromosome band 12q15, which often disrupt the high-mobility-group protein gene HMGIC. In this report, we describe the cytogenetic analysis of an unusual giant cardiac lipoma that exhibited myocardial invasion in a patient with a history of multiple lipomatosis (cutaneous lipoma, lipomatous gynecomastia, lipomatous hypertrophy of the interatrial septum, and dyslipidemia). Cytogenetic studies of cells derived from the cardiac lipoma demonstrated no abnormalities of chromosome 12, but did reveal a t(2;19)(p13;p13.2). A liposarcoma-derived oncogene (p115-RhoGEF) previously mapped to chromosome 19 and the low-density lipoprotein receptor gene (LDLR) previously mapped to chromosome band 19p13 were evaluated to determine whether they were disrupted by this translocation. fluorescence in situ hybridization analyses assigned p115-RhoGEF to chromosome 19 in bands q13.2-q13.3 and mapped the LDLR to chromosome arm 19p in segment 13.2, but centromeric to the t(2;19) breakpoint. Thus, these genes are unlikely to be involved in the t(2;19)(p13;p13.2). Further studies of the regions of chromosomes 2 and 19 perturbed by the translocation in this unusual infiltrating cardiac lipoma will identify gene(s) that participate in adipocyte growth and differentiation and may provide insight into syndromes of multiple lipomatosis.- - - - - - - - - - ranking = 0.625keywords = giant (Clic here for more details about this article) |
6/49. Giant papillary fibroelastoma of the right atrium: an unusual presentation.Papillary fibroelastomas are small tumors of the valvular endocardium with a propensity to embolize. Fibroelastomas originating in the nonvalvular endocardium are rare. We report a giant papillary fibroelastoma of the right atrial septum presenting with hemodynamic compromise that resolved after surgical excision. The current literature and the diagnostic and therapeutic strategies are reviewed.- - - - - - - - - - ranking = 0.125keywords = giant (Clic here for more details about this article) |
7/49. Giant metastatic myxoid liposarcoma causing cardiac tamponade: a case report.We report a case of a rapidly progressing isolated giant metastatic myxoid liposarcoma to the heart in a 54-year-old man, who presented with acute symptoms of cardiac tamponade. Surgery remains the best treatment option for this rare condition.- - - - - - - - - - ranking = 0.125keywords = giant (Clic here for more details about this article) |
8/49. Giant fibroma in the left ventricle of an infant: imaging findings in magnetic resonance imaging, echocardiography and angiography.The diagnosis of a rare case of giant intraventricular fibroma in an infant by MRI in comparison with other imaging modalities, such as echocardiography and angiography, is discussed. For preoperative planning only MRI showed the myocardial infiltration. The myocardial blood supply of the tumor could be evaluated qualitatively by contrast-enhanced MRI, but the direct visualization of the distally located branches could only be assessed by coronary angiography. Magnetic resonance coronary angiography using the navigator technique failed to depict the distal part of the coronaries. The obstruction of the left ventricle outflow tract could be assessed and quantified by all imaging modalities.- - - - - - - - - - ranking = 0.125keywords = giant (Clic here for more details about this article) |
9/49. Giant fibroma of the right ventricle.Cardiac fibromas are rare benign tumours of connective tissue that occur most frequently in children within the left ventricle. Spontaneous regression has not been observed, and surgical intervention is usually required. We have successfully treated a 1-year old girl with a giant fibroma of the right ventricle using the principles of the Batista procedure. The diagnosis was primarily made using transthoracic echocardiography.- - - - - - - - - - ranking = 0.125keywords = giant (Clic here for more details about this article) |
10/49. Electrocardiographic changes in patients with cardiac rhabdomyomas associated with tuberous sclerosis.BACKGROUND: Cardiac rhabdomyomas associated with tuberous sclerosis induce various abnormalities in the electrocardiogram. Electrocardiographic evidence of ventricular hypertrophy may appear if the tumour is electrically active. To our knowledge, electrocardiographic evidence of ventricular hypertrophy has been reported only in association with congestive heart failure. follow-up studies of changes in electrocardiographic findings are also lacking. methods: We studied 21 consecutive patients with cardiac rhabdomyoma associated with tuberous sclerosis, 10 males and 11 females, aged from the date of birth to 9 years at diagnosis. The mean period of follow-up was 53 months. None of the patients developed congestive heart failure. We evaluated the electrocardiographic changes during the follow-up, and their association with echocardiographic findings. RESULTS: Of the 21 patients, 12 showed one or more abnormalities on the electrocardiogram at presentation, with five demonstrating right or left ventricular hypertrophy. In all of these five cases, the tumours were mainly located in the respective ventricular cavity. In one patient with a giant tumour expanding exteriorly, there was marked left ventricular hypertrophy on the electrocardiogram. Followup studies showed spontaneous regression of the tumours in 12 of 19 patients, with abnormalities still present in only 7 patients. A gradual disappearance of left ventricular hypertrophy as seen on the electrocardiogram was noted in the patient with marked left ventricular hypertrophy at presentation in parallel with regression of the tumour. CONCLUSIONS: The presence of cardiac rhabdomyomas in patients with tuberous sclerosis might explain the ventricular hypertrophy seen on the electrocardiogram through its electrically active tissue without ventricular pressure overload or ventricular enlargement, although pre-excitation might affect the amplitude of the QRS complex. Even in cases with large tumours, nonetheless, the electric potential might- not alter the surface electrocardiogram if the direction of growth of the tumour is towards the ventricular cavity. In many cases, electrocardiographic abnormalities tend to disappear, concomitant with regression of the tumours.- - - - - - - - - - ranking = 0.125keywords = giant (Clic here for more details about this article) |
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