Cardiac myxoma:Morphologic, histochemical, and tissue culture studies

Tanimura, Akira; Kitazono, Masatomo; Nagayama, Koji; Tanaka, Syunichi; Kosuga, K

doi:10.1016/s0046-8177(88)80525-2

Cited by 34 publications

(15 citation statements)

References 10 publications

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“…Sarcomas, like myxomas, are presumed to be mesenchymally derived ''stromal'' tumors based on the presence of markers such as vimentin, and some myxoid sarcomas and myxomas (e.g., intramuscular myxomas) that exhibit hypercellular regions are difficult to distinguish histologically (28,29). Moreover, others have invoked a relationship of vascular proliferative lesions to myxomas because myxoma cells can express ''endothelial'' molecular markers (29)(30)(31). CNC's relationship to hepatocellular carcinoma is less clear; PRKAR1␣ was initially identified as a locus that repressed transcription specifically in hepatocellular carcinoma cells (32,33).…”

Section: Discussionmentioning

confidence: 99%

Comparative PRKAR1A genotype–phenotype analyses in humans with Carney complex and prkar1a haploinsufficient mice

Veugelers

Wilkes

Burton

et al. 2004

Proc. Natl. Acad. Sci. U.S.A.

147

122

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Carney complex (CNC) is a familial multiple neoplasia syndrome characterized by cardiac and extracardiac myxomas in the setting of spotty skin pigmentation and endocrinopathy. We previously identified PRKAR1A (regulatory subunit 1α of protein kinase A) mutations in CNC. Mutational analyses of the PRKAR1A gene in 51 unrelated CNC probands now detect mutations in 65%. All mutations, except for one unique missense mutation, lead to PRKAR1A haploinsufficiency. Therefore, we studied the consequences of prkar1a haploinsufficiency in mice. Although we did not observe cardiac myxomas or altered pigmentation in prkar1a +/– mice, we did observe some phenotypes similar to CNC, including altered heart rate variability. Moreover, prkar1a +/– mice exhibited a marked propensity for extracardiac tumorigenesis. They developed sarcomas and hepatocellular carcinomas. Sarcomas were frequently associated with myxomatous differentiation. Tumors from prkar1a +/– mice did not exhibit prkar1a loss of heterozygosity. Thus, we conclude that although PRKAR1A haploinsufficiency does predispose to tumorigenesis, distinct secondary genetic events are required for tumor formation.

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Section: Discussionmentioning

confidence: 99%

Comparative PRKAR1A genotype–phenotype analyses in humans with Carney complex and prkar1a haploinsufficient mice

Veugelers

Wilkes

Burton

et al. 2004

Proc. Natl. Acad. Sci. U.S.A.

147

122

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“…The histogenesis of the tumor is poorly understood. Immunocytochemical studies have identified several differentiation patterns as shown by expression of endothelial, smooth muscle and epithelial markers [6,11,13,19,22,25,29,30,40,46,56]. Recently, cardiomyogenic differentiation [24] and calretinin expression [48] have been demonstrated in the tumor cells, suggesting an origin from a mesenchymal cardiomyocyte progenitor cell or a relationship to endocardial sensory nerve tissue, respectively.…”

Section: Introductionmentioning

confidence: 99%

Cytokeratin profile and neuroendocrine cells in the glandular component of cardiac myxoma

et al. 2003

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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.

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“…Previous studies to identify the cells of origin of cardiac myxomas using immunohistochemistry, electron microscopy, and tissue culture have yielded conflicting results. [3][4][5][6][7] Differentiation toward endothelial, [3][4][5][6] fibroblastic, 5 and smooth muscle cells, 3,5 along with typical stromal cells, has been identified in cardiac myxoma, and glandular differentiation has rarely been observed. 5,7 Neurogenic differentiation has also been suggested based on the findings of S100 protein and neuron-specific enolase immunoreactivity in some tumor cells.…”

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confidence: 99%

“…The cells giving rise to the tumors are considered to be mesenchymal cells that persist as embryonal residues 4 -7 during septation of the heart and may later differentiate into cardiomyocyte precursor cells along with endothelial, [3][4][5][6] fibroblastic, 5 neurogenic, [5][6][7] and smooth muscle cells. [3][4][5] We agree that the identification of tumor origin by specific antigen expression may be misleading, because the phenotypic expression in neoplasia can be variable and does not necessarily reflect its origin. However, the positivity for various cardiomyocyte-specific transcription factors in myxoma cells clearly suggests that cardiac myxoma is a neoplasm arising from mesenchymal cells with a cardiomyogenic lineage.…”

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confidence: 99%

Cardiomyogenic Differentiation in Cardiac Myxoma Expressing Lineage-Specific Transcription Factors

Kodama

Hirotani

Suzuki

et al. 2002

The American Journal of Pathology

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We investigated five cases of cardiac myxoma and one case of cardiac undifferentiated sarcoma by light and electron microscopy, in situ hybridization, immunohistochemical staining, and reverse transcriptase-polymerase chain reaction for cardiomyocyte-specific transcription factors, Nkx2.5/Csx, GATA-4, MEF2, and eHAND. Conventional light microscopy revealed that cardiac myxoma and sarcoma cells presented variable cellular arrangements and different histological characteristics. Ultrastructurally, some of the myxoma cells exhibited endothelium-like or immature mesenchymal cell differentiation. Immunohistochemistry for Nkx2.5/ Csx, GATA-4, and eHAND was slightly to intensely positive in all myxoma cases. MEF2 immunoreactivity was observed in all cases including the case of sarcoma, thus suggesting myogenic differentiation of myxoma or sarcoma cells. In situ hybridization for Nkx2.5/Csx also revealed that all myxoma cells, but not sarcoma cells, expressed mRNA of the cardiac homeobox gene, Nkx2.5/Csx. Furthermore, nested reverse transcriptasepolymerase chain reaction from formalin-fixed, paraffin-embedded tissue was performed and demonstrated that the Nkx2.5/Csx and eHAND gene product to be detected in all cases, and in three of six cases, respectively. In conclusion, cardiac myxoma cells were found to express various amounts of cardiomyocyte-specific transcription factor gene products at the mRNA and protein levels, thus suggesting cardiomyogenic differentiation. These results support the concept that cardiac myxoma might arise from mesenchymal cardiomyocyte progenitor cells. Primary tumors of the heart are quite rare; however, among them cardiac myxomas are the most common ones.

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Cardiac myxoma:Morphologic, histochemical, and tissue culture studies

Cited by 34 publications

References 10 publications

Comparative PRKAR1A genotype–phenotype analyses in humans with Carney complex and prkar1a haploinsufficient mice

Comparative PRKAR1A genotype–phenotype analyses in humans with Carney complex and prkar1a haploinsufficient mice

Cytokeratin profile and neuroendocrine cells in the glandular component of cardiac myxoma

Cardiomyogenic Differentiation in Cardiac Myxoma Expressing Lineage-Specific Transcription Factors

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