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Presented by Ralph Hruban, M.D. and prepared by Shiyama Mudali, M.D.
Case 6: This patient with syncope was found to have an atrial mass on echocardiography.
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1. Question
Week 442: Case 6
This patient with syncope was found to have an atrial mass on echocardiography.images/1alex/07122010case6image1.jpg
images/1alex/07122010case6image2.jpg
images/1alex/07122010case6image3.jpgCorrect
Answer: Atrial myxoma
Histology: This lesion has abundant myxoid stroma. Embedded within the stroma are irregular stellate cells. Unusual appearing vessels can also be seen.
Discussion: Most atrial myxomas arise in the region of the region of the fossa ovalis in the septum of the left atrium of the heart. The patients tend to be middle age and female. There is an association with Carney’s Complex, an autosomal dominantly inherited syndrome characterized by spotty pigmentation, endocrine hyperactivity, and cardiac myxoma. Carney’s Complex is caused by mutations in the PRKAR1 gene on chromosome 17 (a gene that codes for a protein with protein kinase A regulatory function). By light microscopy myxomas are composed of stellate cells floating within an abundant acid mucopolysaccharide ground substance.
Incorrect
Answer: Atrial myxoma
Histology: This lesion has abundant myxoid stroma. Embedded within the stroma are irregular stellate cells. Unusual appearing vessels can also be seen.
Discussion: Most atrial myxomas arise in the region of the region of the fossa ovalis in the septum of the left atrium of the heart. The patients tend to be middle age and female. There is an association with Carney’s Complex, an autosomal dominantly inherited syndrome characterized by spotty pigmentation, endocrine hyperactivity, and cardiac myxoma. Carney’s Complex is caused by mutations in the PRKAR1 gene on chromosome 17 (a gene that codes for a protein with protein kinase A regulatory function). By light microscopy myxomas are composed of stellate cells floating within an abundant acid mucopolysaccharide ground substance.
