Cases reported "Heart Neoplasms"

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1/40. cardiac tamponade originating from primary gastric signet ring cell carcinoma.

    A 45-year-old man with dry cough and dyspnea was referred by a medical practitioner for evaluation of heart failure on February 10, 1996. Chest X-ray revealed increased cardiothoracic ratio, and ultrasonographic echocardiography disclosed massive pericardial effusion with right ventricular collapse. cardiac tamponade was diagnosed and pericardiocentesis was performed. Ten days after admission, the pleural effusion had become more pronounced, and thoracocentesis was performed. carcinoembryonic antigen level was elevated in both the pericardial and pleural effusion, and cytology implicated adenocarcinoma, which suggested malignant effusion. Endoscopic study disclosed gastric cancer in the posterior wall of the upper body, and the histopathological diagnosis was signet-ring cell carcinoma. The patient died of respiratory failure on May 2, 1996, and autopsy was performed. The final diagnosis was gastric cancer with pulmonary lymphangitis, pericarditis, and pleuritis carcinomatosa, accompanied by enlargement of mediastinal and paraaortic lymph nodes. Interestingly, the primary signet-ring cell carcinoma of the stomach was situated mostly in the mucosa. Deep in the submucosal region, there was prominent invasion of the intralymphatic vessels, without direct destruction of the mucosa muscularis.
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2/40. An autopsy case of a malignant pericardial mesothelioma in a Japanese young man.

    An autopsy case of a malignant pericardial mesothelioma in a 27-year-old man with no history of exposure to asbestos is reported. He was admitted for heart failure due to pericardial effusion of unknown origin and surgically drained, but later died. The diagnosis of a malignant pericardial mesothelioma was made on the basis of histologic, immunohistochemical and ultrastructural findings. The tumor was located on the pericardium, but autopsy revealed that it had spread extensively in the mediastinum and the lungs. Microscopically, the tumor cells were epithelial like and contained histochemically demonstrable glycogen and hyaluronic acid. Immunohistochemical studies of the tumor demonstrated positive immunoreactivity for cytokeratin 19, muscle actin HHF35, epithelial membrane antigen, CA125, p53 and p21WAF1/CIP1 whereas the tumor was negative for cytokeratins 10 and 17, carcinoembryonic antigen, vimentin, epithelial antigen BerEP4, S-100, c-erbB2 and bcl-2. A high MIB-1 labeling index was noted. Under the electron microscope the tumor cells exhibited long, thin villi. The operation and autopsy findings thus revealed this to be a very rare case of malignant pericardial mesothelioma in a young man.
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3/40. Vacuolated cell mesothelioma of the pericardium resembling liposarcoma: a case report.

    We report a case of localized pericardial mesothelioma with unusual histological features in a 44-year-old woman. Her radiological imagings showed an 11-cm pericardial tumor, between the heart and aortic arch. Microscopically, the tumor was predominantly composed of vacuolated cells and vaguely reminiscent of well differentiated "lipoma-like" liposarcoma, but only small foci of the tumor showed the papillotubular configuration. Histochemically, the tumor cells contained hyaluronic acid in the vacuoles but no lipids. Immunohistochemically, they showed immunoreactivity for cytokeratin, calretinin, vimentin, and epithelial membrane antigen. Ultrastructural study showed that the vacuoles of the tumor cells were intracytoplasmic lumina. The intracytoplasmic lumina and the surface membranes of the tumor cells had many long and slender microvilli with focal bush-like appearance. desmosomes between adjacent cells were occasionally observed. To our knowledge, this is the first case report of epithelial type mesothelioma predominantly composed of vacuolated tumor cells, microscopically mimicking liposarcoma.
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4/40. A primary cardiac sarcoma with unusual histology and clinical course.

    A left atrial tumor, in which radical resection was impossible, demonstrated two processes: An inflammatory pseudotumor and cellular atypia suggestive of a sarcoma. immunohistochemistry (proliferating cell nuclear antigen [PCNA], MIB-1 [Ki-67 antibody], bcl-2 positive; p53 negative, focal loss of nm23) was supportive for a malignant tumor. Despite no further therapy because of uncertainty in tumor classification, the patient remained in remission for 28 months. Thereafter, spine metastases and local regrowth were found, and the patient died 15 months later, after temporary remission by radiotherapy. This case stresses the impact immunohistochemistry may have on diagnosis of malignancy and the difficulty in predicting the biological behavior of cardiac sarcomas.
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5/40. Pediatric angiosarcoma of the heart: a unique presentation and metastatic pattern.

    We report the seventh case of angiosarcoma of the heart in a child. The patient was a 23-month-old female who presented for lower extremity limping and underwent open surgical biopsy of the femur. Immediately postoperatively, she developed pericardial tamponade, and a bulky intracardiac mass was discovered as the underlying cause. The mass was composed of highly pleomorphic tumor cells reactive for the endothelial markers CD31, CD34, and factor viii-related antigen (FVIII-RA). Staging evaluation revealed widespread metastases involving the brain, ovaries, and bone marrow. She died of complications of metastatic disease 8 months following initial presentation. Unusual features of this case include the young age of the patient, left-sided nature of the cardiac tumor, presentation secondary to metastatic disease, and the pattern of metastases. The literature on cardiac angiosarcoma, which is limited to six case reports in the pediatric population, is also reviewed.
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6/40. Primary epithelial malignant mesothelioma of the pericardium with deciduoid features: cytohistologic and immunohistochemical study.

    Malignant mesothelioma with deciduoid features (MMWDF) is a recently characterized morphologic variant of epithelioid malignant mesothelioma, which frequently is misdiagnosed as peritoneal deciduosis or florid mesothelial hyperplasia. We report on the cytological, histological, immunohistochemical, and autopsy findings of a case of MMWDF arising in the pericardium of a 71-yr-old female patient. Cytology showed large, polygonal to round cells with pale to bright, eosinophilic cytoplasm, occasionally showing xantomatous pattern, containing a pleomorphic and vesicular nucleus with a single prominent nucleolus. autopsy examination showed a neoplasm encasing the heart and great vessels. No other primary neoplasm was found. The histological analysis disclosed the typical features of MMWDF. immunohistochemistry showed diffuse immunoreactivity for cytokeratin MNF116, HBME-1, and calretinin in the neoplastic cells, as well as focal positivity for epithelial membrane antigen positivity in a brush border-like pattern. All other markers were negative. We would like to stress that pathologists must be aware of the cytological and histological features of this rare variant of epithelioid malignant mesothelioma in order to avoid a misdiagnosis of a benign process or a metastatic malignancy.
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7/40. Tumorous deformity of mitral valve leaflet after chordal rupture in a child.

    A case with tumorous deformity of the posterior mitral valve leaflet after spontaneous chordal rupture in a child is described. A partial rupture in the chordae tendineae of the posterior mitral leaflet was found by echocardiography in a 9-year-old Japanese boy. Tumorous bulging was gradually developed in the leaflet and was surgically excised 5 years later. Multiple nodular tumors were found on the atrial surface of the posterior mitral leaflet. Histological examination revealed that the tumorous bulging consisted of myxomatous materials in which collagen fibrils and very fine elastic fibers were distributed loosely and irregularly. Normal-looking endothelial cells covered the luminal surface of the bulging lesion. vimentin-positive spindle-shaped mesenchymal cells were scattered in the bulge area. The labeling index of proliferating cell nuclear antigen (PCNA) in these cells was 29.3%. These spindle cells were positive for matrix metalloproteinase (MMP)-1 in the entire bulge area. The cells and matrix were positive for MMP-2 and tissue inhibitor of MMP (TIMP)-1 in the basal area of bulging, but were weakly positive or negative at the surface area. Reactivity for TIMP-2 in the cells in the bulge area was obviously weaker than that in the cells at the spongiosa of the anterior mitral leaflet, which was obtained from the patient at the valve replacement operation 9 months after the initial operation. These findings indicated that the tumorous deformity of the mitral valve was formed by the overgrowth of valve tissue under the stimulation of mitral regurgitation in this child, and the imbalance of MMP and TIMP might play an important role in the bulge formation.
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8/40. Cardiac myxoma with a cytokeratin-immunoreactive glandular component.

    This study documents the expression of cytokeratin intermediate filaments (IFs) in a surgically excised glandular atrial myxoma. The glandular structures showed also positivity for carcinoembryonic antigen. Typical isolated or cordlike myxoma cells contained vimentin IFs. Positivity for von willebrand factor was detected in cells lining deep invaginations of surface papillae and vascular channels. Bundles of smooth muscle cells and myofibroblasts scattered throughout the myxoid matrix synthesized the isoform of alpha-actin specific for smooth muscle.
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9/40. Mobilization of the posterior leaflet of the mitral valve for resection of a left ventricular tumor producing carbohydrate antigen 19-9.

    We report an extremely rare case of a left ventricular tumor producing carbohydrate antigen 19-9 (CA19-9). A 43-year-old man with an episode of right hemiplegia underwent an emergent operation for a left ventricular tumor just beneath the posterior leaflet of the mitral valve. The tumor was successfully resected by mobilization of the posterior leaflet of the mitral valve. The resected specimen was immunoreactive for CA19-9.
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10/40. Primary cardiac angiosarcoma with systemic metastases; a case report and review of the literature.

    Angiosarcoma is a relatively uncommon primary malignant tumor of the heart derived from the endothelial cells. The authors report a case of primary cardiac angiosarcoma of the right atrium with pulmonary, hepatic, cerebellar and bony metastases in a 46-year-old man who clinically presented as pulmonary hemorrhage. The patient's occupational history had occasional exposure to polyvinyl chloride. The autopsy examination of the cardiac angiosarcoma characterized macroscopically by a right atrial tumor and histologically by anastomosing vascular channels and minute atypical pleomorphic endothelial cells. Immunohistological stainings were positive CD31, CD34 and focal positive factor VII-related antigen. Clinical and pathologic features with briefly reviewed relevant literatures are discussed This is the first reported description in the literature of a primary cardiac angiosarcoma with systemic metastases to multiple organs in thailand.
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