Benign Myxoma Research Articles

Primary cardiac lymphoma: molecular cytogenetic characterization of a rare entity

The majority of cardiac atrial neoplasms represent benign myxomas. Rarely, malignant cardiac neoplasms are encountered and can include primary cardiac neoplasms, as well as secondary tumors involving the heart. As many cardiac neoplasms lack pathognomonic clinical features, histopathologic diagnosis is crucial for classification and appropriate treatment of these neoplasms. Molecular investigation is critical to begin to catalogue genomic changes that correlate with these malignancies. A 60-year-old man presented with superior vena cava syndrome, and computed tomographic scan revealed an infiltrative mass of the right atrium that nearly filled the atrial chamber and partially occluded superior vena cava flow. Urgent surgical resection revealed a soft mass with the appearance of "fish flesh." Histologic, immunochistochemical, cytogenetic, and detailed molecular investigations were carried out. Histologic examination revealed complete replacement of the atrial wall by diffuse sheets of pleomorphic lymphoid cells with occasional smaller plasmacytoid cells. The predominant lymphoid population was immunoreactive for CD45, CD20, CD79a, BCL-2, BCL-6, Ki-67, CD10, p53, and light chain restricted for IgM lambda. A diagnosis of primary cardiac diffuse large B-cell lymphoma with plasmacytoid differentiation was established and was supported by cytogenetic studies demonstrating the presence of a t(14;18)(q32;q21) translocation in addition to other chromosomal abnormalities. Fluorescence in situ hybridization revealed no evidence of a C-MYC translocation. In this single case, comparative genomic hybridization analysis using both bacterial artificial chromosome and oligonucleotide arrays correlated well with cytogenetic findings and allows for the cataloguing of more subtle genomic events.

Benign cardiac tumor in a patient with lung malignancy

Dr. Jochen W.U. Fries Institute of Pathology University of Cologne Kerpener Str. 62 50937 Cologne, Germany Sirs: With an incidence of approximately 0.02% in autopsy series, primary tumors of the heart are very rare [15]. While 80–90% of primary cardiac neoplasms seen in the left atrium are benign myxomas, tumors of the right side of the heart are always suspicious of malignancy [15]. Metastatic cardiac tumors are 20 to 40 times more common than primary heart neoplasms and have been reported in up to 20.6% of patients with extracardiac malignancies [7]. In most of these patients cardiac involvement is a sign of poor prognosis. We present a patient with squamous cell lung cancer and a concomitant huge right-atrial mass, in whom precise cardiac evaluation and subsequent histopathological diagnosis were essential for further therapy.

Pseudomyxoma Originating from the Interatrial Septum in a Heart Transplant Patient

This report describes the echocardiographic diagnosis of an intracardiac mass in a heart transplant patient. The typical morphology of the lesions, its attachment to the interatrial septum, and the absence of a nidus focus for thrombus formation led to the preoperative assumption that it was a myxoma. The mass was successfully excised at the time of operation and the patient had an uneventful recovery. Pathology findings revealed a thrombus. Thus, echocardiography may have limited specificity for the differentiation of intracardiac tumors and thrombus.

CT for intracardiac thrombi and tumors

Although cardiac tumors are rare, they nevertheless represent an important subgroup, the diagnosis of which is challenging for the primary care physician. Symptoms are not characteristic and serious complications including stroke, myocardial infarction and even sudden death from arrhythmia may be the first signs of tumor. The most common primary cardiac neoplasm is the benign myxoma and the most frequent primary malignant lesion is sarcoma. Cardiac metastases from distant primary carcinomas are now frequently encountered. Echocardiography until the past decade was the only consistently reliable and available non-invasive diagnostic tool. New non-invasive CT and MRI exams are changing the diagnostic approach. Echocardiography and angiocardiography with or without coronary arteriography remain routine methods for evaluating cardiac neoplasms. However, CT provides additional diagnostic information and is regarded as essential for adequate staging and treatment planning, particularly when surgical resection is being considered. This chapter reviews the wide spectrum of intracardiac thrombi and tumors including their incidence and imaging characteristics with particular reference to the CT findings and differential diagnosis.

Right atrial appendage myxoma following recent coronary artery bypass grafting

A 71-year-old male presented with recurrent atrial fibrillation, anaemia and thrombocytopenia. Six months ago he underwent an urgent triple coronary artery bypass-grafting elsewhere. Postoperatively he complained of fatigue and low-grade fever. Echocardiographs and magnetic resonance imaging showed a right atrial appendage mass, which afterwards was resected. Histology confirmed a benign myxoma. Patient's symptoms spontaneously resolved. This report demonstrates the unusual sites that myxoma may occur, with probable serious complications in case of urgent heart operations. This case also emphasizes the need for preoperative echocardiography in open heart operations or in cases with recurrent atrial fibrillation.

Minichromosome maintenance protein in myxofibrosarcoma

Histopathological assessment of myxofibrosarcoma may be difficult, especially on the basis of a small core biopsy, which enables only a crude evaluation of grade and prognosis. We have tested the hypothesis that determination of cell cycle state may assist in the diagnostic assessment of myxofibrosarcoma. We have studied 51 cases of high-grade (n=20), intermediate-grade (n=21), and low-grade (n=10) myxofibrosarcomas, as well as nine cases of benign myxoma. Cell cycle state within tumors was determined by immunostaining for the recently described marker minichromosome maintenance protein 2 (MCM2), together with Ki67. Labelling indices for both markers were correlated with tumor grade, mitotic index, and time to first recurrence. The MCM2 labelling indices were significantly higher than the Ki-67 labelling indices. Both indices showed a significant correlation with the mitotic index and both showed significant increases with increasing grade of myxofibrosarcoma. The MCM2 labelling index (but not the Ki67 labelling index) showed a significant inverse exponential correlation with the time to first recurrence. Myxoid and cellular areas showed no difference in the MCM2 and Ki-67 labeling index, suggesting that clinically useful information could be obtained from any component of a myxofibrosarcoma sampled in a needle biopsy and/or cytological specimen. We therefore suggest that assessment of cell cycle state may be a useful diagnostic adjunct in the histopathological assessment of myxofibrosarcoma, by enabling more accurate determination of grade and prediction of outcome.

Anesthetic Management for the Excision of Left Atrial Myxoid Sarcoma Preoperatively Diagnosed as a Left Atrial Myxoma: A case report

We report a case of myxoid sarcoma in the left atrium, which was diagnosed as benign myxoma on preoperative echocardiography. At operation, semisolid mobile masses were found that attached with broad stalk to the posterior left atrial wall and that invaded into the left pulmonary vein and the anterior mitral leaflet. The patient underwent excision of left atrial tumor masses, mitral valve replacement, and left pneumonectomy. The histological diagnosis was undifferentiated cardiac myxoid sarcoma. Although echocardiography lead to the choice of diagnostic test of intra-atrial tumor, magnetic resonance imaging, computed tomography, and intraoperative transesophageal echocardiography may be needed for differential diagnosis, with indeterminate malignant findings on preoperative transthoracic echocardiography. Anesthesiologists are often confronted with cases that are unusual, therefore preoperative understanding of the cases are important for safe anesthetic management.

Production of Endothelin-1 and Big Endothelin-1 by Human Cardiac Myxoma Cells-Implications for the Origin of Myxomas-

Although the origin of cardiac myxomas is still controversial, the 2 main hypotheses are that the tumor cells originate either from multipotential mesenchymal cells or from endocardial neural tissue. The production of various cytokines in 2 human cardiac myxoma cell lines was examined by enzyme-linked immunosorbent assay. After 7 days of culture, extremely high concentrations of interleukin-6 were detected in the culture media from both myxoma cell lines. Increased production of CXC chemokines, interleukin-8 and growth-related oncogene-alpha, were observed in both myxoma cell lines. Endothelin (ET)-1 and its precursor, big ET-1, were detected in the culture media from both myxoma cell lines. The production of both ET-1 and big ET-1 by myxoma cells was higher than by human umbilical vein endothelial cells. Similar to endothelial cells, myxoma cells did not produce stem cell factor, granulocyte colony-stimulating factor, hepatocyte growth factor, or ET-3. The similarity of the cytokine production pattern between cardiac myxoma cells and endothelial cells supports the hypothesis that the tumor cells originate from mesenchymal cells capable of endothelial differentiation. Overproduction of CXC chemokines may explain, in part, the malignant potential of histologically benign myxomas.

Vascular endothelial growth factor is an autocrine growth factor for cardiac myxoma cells.

Cardiac myxomas are generally considered benign, but malignant tumors have been reported. Vascular endothelial growth factor (VEGF), an angiogenic factor, plays a role in the growth, progression, and metastasis of solid tumors and it has been reported that VEGF expression is upregulated in cardiac myxomas that have a high microvessel density. The purpose of this study was to determine whether cardiac myxoma cells possess a VEGF-autocrine system that regulates tumor growth. Immunohistochemical analyses revealed the presence of VEGF and its receptors, VEGFR-1 (flt-1) and VEGFR-2 (KDR/flk-1), in the cytoplasm of tumor cells from 18 of 18 myxoma tissue specimens examined. Two different myxoma cell lines were established and constitutively secreted large amounts of VEGF as determined by enzyme-linked immunosorbent assay. The expression of VEGF, VEGFR-1, and VEGFR-2 mRNA was detected in both cell lines by reverse-transcriptase polymerase chain reaction. Myxoma cell proliferation, as determined by thymidine incorporation, was enhanced by the addition of VEGF in a dose-dependent manner, and cell proliferation was inhibited in a dose-dependent manner by the addition of a neutralizing VEGF antibody. These results indicate that cardiac myxoma cells possess a VEGF-autocrine system, which could contribute to the malignant potential of histologically benign myxomas through direct stimulation of tumor cell growth as well as through induction of angiogenesis.

Minichromosome maintenance protein in myxofibrosarcoma

Histopathological assessment of myxofibrosarcoma may be difficult, especially on the basis of a small core biopsy, which enables only a crude evaluation of grade and prognosis. We have tested the hypothesis that determination of cell cycle state may assist in the diagnostic assessment of myxofibrosarcoma. We have studied 51 cases of high-grade (n=20), intermediate-grade (n=21), and low-grade (n=10) myxofibrosarcomas, as well as nine cases of benign myxoma. Cell cycle state within tumors was determined by immunostaining for the recently described marker minichromosome maintenance protein 2 (MCM2), together with Ki67. Labelling indices for both markers were correlated with tumor grade, mitotic index, and time to first recurrence. The MCM2 labelling indices were significantly higher than the Ki-67 labelling indices. Both indices showed a significant correlation with the mitotic index and both showed significant increases with increasing grade of myxofibrosarcoma. The MCM2 labelling index (but not the Ki67 labelling index) showed a significant inverse exponential correlation with the time to first recurrence. Myxoid and cellular areas showed no difference in the MCM2 and Ki-67 labeling index, suggesting that clinically useful information could be obtained from any component of a myxofibrosarcoma sampled in a needle biopsy and/or cytological specimen. We therefore suggest that assessment of cell cycle state may be a useful diagnostic adjunct in the histopathological assessment of myxofibrosarcoma, by enabling more accurate determination of grade and prediction of outcome.

Successful surgical treatment of a right atrial myxoma complicated by pulmonary embolism

We report on a rare case of a 65-year-old woman who was admitted with orthopnea (NYHA class IV) and a single syncopal episode. A transthoracic echocardiography examination showed a 7x6x6-cm mass located in the right atrium and perfusion lung scan showed embolization. Complete surgical removal of the right atrial myxoma and tumor embolectomy was successfully performed with deep hypothermia and circulatory arrest. Histologic investigations of the primary tumor as well as the emboly revealed benign myxoma. Six months clinical and echocardiographical follow-up showed a satisfactory exercise tolerance, a normal right atrium and a good ventricular function. Thirteen years later, the patient remains well and no recurrence could be observed.

Calcified Left Ventricular Mass: Unusual Clinical, Echocardiographic, and Computed Tomographic Findings of Primary Cardiac Osteosarcoma

Calcified Left Ventricular Mass: Unusual Clinical, Echocardiographic, and Computed Tomographic Findings of Primary Cardiac Osteosarcoma

Malignant Fibrous Histiocytoma of the Heart: Case Report and Literature Review

A case report of a 57-year-old woman with malignant fibrous histiocytoma of the heart is presented. The clinical presentation was suggestive of a benign atrial myxoma. Careful pathologic study is necessary to differentiate the myxoid variant of malignant fibrous histiocytoma of the heart from the more common benign atrial myxoma. Usually, the evolution of malignant fibrous histiocytoma of the heart is rapidly fatal. Clinical aspects of this patient's case are discussed in conjunction with a review of the available literature.

A cardiac myxoma with interleukin-6 production and cerebral metastasis

The occurrence of cerebral metastasis from cardiac myxoma is extremely rare. We present a 70-year-old man who had atrial myxoma with two metastatic myxomas in his brain. Histopathologic examination revealed a benign myxoma. An elevated interleukin-6 (IL-6) concentration in the serum, and immunohistochemical staining of the primary myxoma by an anti-IL-6 monoclonal antibody confirmed interleukin production by the myxoma. The IL-6-positive myxoma cells were closely attached to the cerebral endothelial cells. This suggests that IL-6 production by the atrial myxoma may have been an important factor for intravascular metastasis or embolization.

Cardiac Myxoma Metastasized to the Brain: Potential Role of Endogenous lnterleukin-6

Metastasis of a cardiac myxoma to the brain is extremely rare. We present the case of a 70-year-old man who had an atrial myxoma and two metastatic myxomas in the brain. The intracranial lesions were in fact diagnosed before the cardiac myxoma, since the patient developed hemiparesis before his cardiac symptoms occurred. Computerized tomography of the brain showed two high-density lesions, the larger of which was removed surgically. Follow-up computerized tomography revealed a progressive enlargement of the second lesion which was then resected. Histopathological examination showed all lesions to the benign myxomas. Interestingly, high concentrations of interleukin-6 were present in the patient's serum and cardiac myxoma. Interleukin-6 may possibly potentiate metastasis of cardiac myxoma.

Sequential MaIignant Transformation of Cardiac Myxoma

We describe a case of cardiac myxoma in a 44-year-old Japanese man, who died after developing metastases in the skin, brain and muscle. A satellite tumor which was attached to the wall of the abdominal aorta induced marked hypertension due to obstruction of the renal arteries. Although the primary heart tumor had typical histological features of benign cardiac myxoma, the recurrent heart tumor, which was partly resected three months before the patient's death, showed apparently malignant characteristics resembling malignant fibrous histiocytoma (MFH). Since the histological features of the initial and recurrent tumors were different, the grade of malignancy was investigated using the cellularity of the tumor as an arbitrary criterion. A gradual but significant increase in the cellularity was observed over the course of five years. Immunohistochemically, tumor cells in the muscle metastasis contained vimentin and factor VIII-related antigen, and multinucleated giant cells in the recurrent heart tumor contained desmin, which is rarely detectable in MFH. Therefore, we considered that the present case represented malignant transformation of benign cardiac myxoma.

Tumors of the Heart Experiences at the Giessen University Clinic

Of 54 cardiac tumors operated upon in our clinic, 42 were classified as benign and only 12 as malignant. The major part of the benign tumors were myxoma, mainly located in the left atrium. While smaller tumors could be treated by local resections, extensive resections were necessary in 14 patients with greater tumors followed by reconstructions of the pulmonary and caval vein, mitral and tricuspid valve, and major parts of the right and left ventricular wall. In one patient with a huge benign myxoma, tumor exposition and total resection could only be achieved by an autotransplantation of the heart. While mortality after surgical therapy of benign tumors was only 1.4% (1/42) within a mean follow-up time of 48 months, the prognosis of malignant tumors is still fatal with a mortality of 50% (6/12) within a mean follow-up time of 24 months, despite additional chemotherapy or radiation.

Benign soft-tissue neoplasm simulating sarcoma of spermatic cord

A large, painless, slow-growing mass arising in the scrotum of a twenty-three-yearold man, which proved to be benign myxoma, simulated a primary sarcoma of the spermatic cord. Only I case of myxoma involving the spermatic cord has been reported previously. It is extreme important that multiple microscopic sections of the specimen be examined carefully so that the proper tissue diagnosis can be made. A false diagnosis of malignancy may lead to treatment that not indicated and that has a high morbidity.

Primary cardiac tumours--operative treatment of 20 patients.

This report describes a series of 20 patients operated on for a primary cardiac tumour. The majority of the tumours (16) were benign myxomas; 12 of them were located in the left atrium, two in the right atrium and two were biatrial. Two lipomas were found; one was epicardial and the other was located in the left atrium. The only intraventricular tumour was a malignant left ventricular myosarcoma. The propensity of intracardiac tumours to embolize was distinctive. Nine of the 16 myxomas presented with peripheral embolization, and in two patients surgery was complicated by fatal perioperative cerebral embolization of myxomatous tissue. Furthermore, in one patient embolization of a left atrial lipoma necessitated amputation of her left arm before cardiac surgery. Late postoperative recurrences were found in two patients with atrial myxomas. In one of them, reoperation showed that the tumour had grown at that site in the interatrial septum where the original pedicle had been excised. One patient developed severe mitral valve regurgitation and underwent replacement with a prosthetic valve at reoperation. Otherwise our late follow-up study showed that the results of surgery were usually excellent even though mild echocardiographic abnormalities were not uncommon. Our experience emphasizes the embolic potential of intracardiac myxomas and suggests, furthermore, that to avoid recurrences excisions with wide margins should be preferred. Echocardiography is an optimal method for the follow-up of these patients.

Primary cardiac tumors: case presentations and anesthetic considerations for lesions other than classical benign myxomas

Primary cardiac tumors: case presentations and anesthetic considerations for lesions other than classical benign myxomas