“…There have been only few cases of malignant glandular cardiac myxomas reported [12,38]. Among these, it could be argued that the malignant behavior in at least some was the result of local recurrence of the tumor, tumor multifocality or embolic phenomena [3,33].…”
Glandular cardiac myxomas are very rare tumors of uncertain histogenesis that display glandular structures within otherwise typical myxomatous tissue. The origin of the glands has been attributed to epithelial differentiation of a totipotent cardiomyogenic precursor cell or to entrapped embryonal rests in the tumor. We studied four cases of glandular myxomas (three sporadic and one familial) to define the immunophenotypic profile of the glandular elements. The glands were either single and located within the myxoma cell islands (three cases) or in groups embedded in the myxomatous matrix. In the latter case, the glands featured villous projections, irregular profile, active inflammation or focal reactive cellular atypia (case 3) and had acidic and neutral mucins (mostly sialomucins). The cytokeratin expression profile (cytokeratin 7 and 20 co-expression) was similar to that of foregut derivatives. Scattered chromogranin-positive neuroendocrine cells were observed in case 3. Our findings indicate that the glandular component in cardiac myxoma is morphologically heterogeneous. In some cases, the scattered glands may derive from a divergent (epithelial) differentiation of myxoma cells; in others, entrapment of embryonic gastrointestinal rests (with mature neuroendocrine and mucous cell populations) could be the case.
“…There have been only few cases of malignant glandular cardiac myxomas reported [12,38]. Among these, it could be argued that the malignant behavior in at least some was the result of local recurrence of the tumor, tumor multifocality or embolic phenomena [3,33].…”
Glandular cardiac myxomas are very rare tumors of uncertain histogenesis that display glandular structures within otherwise typical myxomatous tissue. The origin of the glands has been attributed to epithelial differentiation of a totipotent cardiomyogenic precursor cell or to entrapped embryonal rests in the tumor. We studied four cases of glandular myxomas (three sporadic and one familial) to define the immunophenotypic profile of the glandular elements. The glands were either single and located within the myxoma cell islands (three cases) or in groups embedded in the myxomatous matrix. In the latter case, the glands featured villous projections, irregular profile, active inflammation or focal reactive cellular atypia (case 3) and had acidic and neutral mucins (mostly sialomucins). The cytokeratin expression profile (cytokeratin 7 and 20 co-expression) was similar to that of foregut derivatives. Scattered chromogranin-positive neuroendocrine cells were observed in case 3. Our findings indicate that the glandular component in cardiac myxoma is morphologically heterogeneous. In some cases, the scattered glands may derive from a divergent (epithelial) differentiation of myxoma cells; in others, entrapment of embryonic gastrointestinal rests (with mature neuroendocrine and mucous cell populations) could be the case.
“…Some authors also reported malignant characteristics such as local invasion and metastasis [1, 10, 11, 12]. Myxoma may not only lead to distal embolization but also to mitral valve obstruction, which can occur at any time and precipitate sudden death [3].…”
Cardiac myxoma is the most common form of primary heart tumor and often treated with surgical resection without a preoperative angiographic examination for fear of potential risk of sudden death. During the last 14 years, 24 of 38 patients with myxoma underwent coronary angiography. Coronary artery disease (CAD) and other abnormalities were found in 5. Our findings indicated that CAD is not uncommon among the myxoma patients, and coronary angiography should be performed preoperatively in all cases.
“…First, they are not caused by blood-flow dynamics such as berry aneurysms. In all likelihood they are caused by myxomatous tumour invasion into the vessel wall causing its weakening 6. Pathologically proven myxomatous emboli have been demonstrated in cerebral arteries 7.…”
SUMMARYCardiac myxomas are a very uncommon cause of cerebral aneurysms. We present a case of a young woman with neurological symptoms attributed to small cortical infarctions and multiple cerebral aneurysms, caused by a cardiac myxoma.
BACKGROUND
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