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Presented by Risa Mann, M.D. and prepared by Greg Seidel, M.D.
Case 6: 50-year-old male with decreased cardiac output.
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Week 105: Case 6
50-year-old male with decreased cardiac output./images/0923026a.jpg
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/images/0923026e.jpgCorrect
Answer: Cardiac myxoma
Histology: The tumor was a lobulated mass. Microscopically it is composed of abundant loose myxoid tissue with associated spindled stellate cells. The myxoid tissue is associated with fibrin and some fibrosis. There are scattered lymphocytes, plasma cells and hemosiderin deposition. There is no pleomorphism or significant mitotic activity. Some of the spindle cells seem to form cores that are somewhat suggestive of vascular channels.
Discussion: The cells have a characteristic histologic appearance that is seen in this case and is usually easily distinguished from other more aggressive tumors, such as the various sarcomas, which may occur in the heart. Cardiac myxomas may be sporadic or familial. The sporadic tumors more commonly involve the left atrium and often occur in middle-aged women. They are almost always solitary tumors. In contrast, the familial variety are less commonly located in the left atrium, may be multicentric in up to 30% of cases and are slightly more frequent in males. There has been great controversy as to whether cardiac myxomas are true neoplasms vs. an exuberant reaction to organizing thrombus material. Currently, most evidence favors the neoplastic theory. Chromosomal abnormations as well as cases with aneuploid DNA levels have been reported favoring this hypothesis. The cell of origin of this tumor is also controversial. Ultrastructural studies suggest that the tumors arise from mesenchymal cells. The immunohistochemistry has been confusing. Some have reported strong positivity for factor VIII in favor of endothelial origin. Others have found positive staining for factor VIII as well as other antigens such as actin, desmin, smooth muscle myosin, alpha 1 antitryipsin and alpha 1 chymotrypsin. Some cases have shown focal areas of glandular differentiation that have been reported to be positive for CEA, EMA and keratin. These findings would be more consistent with the theory that cardiac myxomas arise from mesenchymal cells that have the ability to show various patterns of differentiation.
Treatment is surgical excision, which is usually curative.
Incorrect
Answer: Cardiac myxoma
Histology: The tumor was a lobulated mass. Microscopically it is composed of abundant loose myxoid tissue with associated spindled stellate cells. The myxoid tissue is associated with fibrin and some fibrosis. There are scattered lymphocytes, plasma cells and hemosiderin deposition. There is no pleomorphism or significant mitotic activity. Some of the spindle cells seem to form cores that are somewhat suggestive of vascular channels.
Discussion: The cells have a characteristic histologic appearance that is seen in this case and is usually easily distinguished from other more aggressive tumors, such as the various sarcomas, which may occur in the heart. Cardiac myxomas may be sporadic or familial. The sporadic tumors more commonly involve the left atrium and often occur in middle-aged women. They are almost always solitary tumors. In contrast, the familial variety are less commonly located in the left atrium, may be multicentric in up to 30% of cases and are slightly more frequent in males. There has been great controversy as to whether cardiac myxomas are true neoplasms vs. an exuberant reaction to organizing thrombus material. Currently, most evidence favors the neoplastic theory. Chromosomal abnormations as well as cases with aneuploid DNA levels have been reported favoring this hypothesis. The cell of origin of this tumor is also controversial. Ultrastructural studies suggest that the tumors arise from mesenchymal cells. The immunohistochemistry has been confusing. Some have reported strong positivity for factor VIII in favor of endothelial origin. Others have found positive staining for factor VIII as well as other antigens such as actin, desmin, smooth muscle myosin, alpha 1 antitryipsin and alpha 1 chymotrypsin. Some cases have shown focal areas of glandular differentiation that have been reported to be positive for CEA, EMA and keratin. These findings would be more consistent with the theory that cardiac myxomas arise from mesenchymal cells that have the ability to show various patterns of differentiation.
Treatment is surgical excision, which is usually curative.
