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Presented by Ralph Hruban, M.D. and prepared by Amy Duffield, M.D., Ph.D.
Case 1: This forty something year-old woman experienced multiple episodes of syncope.
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1. Question
Week 350: Case 1
This forty something year-old woman experienced multiple episodes of syncope.images/4_28_08 1A.jpg
images/4_28_08 1B.jpg
images/4_28_08 1C.jpg
images/4_28_08 1D.jpg
images/4_28_08 1E.jpgCorrect
Answer: Atrial myxoma
Histology: This lesion is intimately attached to the atrial wall. It is composed of an amorphous myxoid stroma in which stellate cells (“lepidic”) are embedded.
Discussion: Myxoma is the most common neoplasm of the heart in adults. (Rhabdomyomas are more common in children). The vast majority of myxomas arise in the atria (90%), and origin in the left side of the heart is more common than origin in the right side of the heart. They often involve the fossa ovalis. Myxomas with a polypoid growth pattern tend to cause congestive heart failure, while those with a villous architecture tend to produce emboli. Histologically, as illustrated by the case presented, myxomas are composed of stellate cells and blood vessels admixed with mucopolysaccaride substance. Epithelial cells forming glands are found in some cases.
A familial clustering of myxomas occurs in individuals with Carney’s Syndrome. Carney’s Syndrome is an autosomal dominant syndrome characterized by cardiac and extracardiac myxomas, spotty skin pigmentation and endocrine over activity. Carney’s syndrome is caused by germ line mutations in the PRKAR1 gene on chromosome 17. The PRKAR1 gene encodes for cAMP-dependent protein kinase A.
Reference(s):
– Casey et al., J Clin Invest. 2000 Sep;106(5):R31-8.Incorrect
Answer: Atrial myxoma
Histology: This lesion is intimately attached to the atrial wall. It is composed of an amorphous myxoid stroma in which stellate cells (“lepidic”) are embedded.
Discussion: Myxoma is the most common neoplasm of the heart in adults. (Rhabdomyomas are more common in children). The vast majority of myxomas arise in the atria (90%), and origin in the left side of the heart is more common than origin in the right side of the heart. They often involve the fossa ovalis. Myxomas with a polypoid growth pattern tend to cause congestive heart failure, while those with a villous architecture tend to produce emboli. Histologically, as illustrated by the case presented, myxomas are composed of stellate cells and blood vessels admixed with mucopolysaccaride substance. Epithelial cells forming glands are found in some cases.
A familial clustering of myxomas occurs in individuals with Carney’s Syndrome. Carney’s Syndrome is an autosomal dominant syndrome characterized by cardiac and extracardiac myxomas, spotty skin pigmentation and endocrine over activity. Carney’s syndrome is caused by germ line mutations in the PRKAR1 gene on chromosome 17. The PRKAR1 gene encodes for cAMP-dependent protein kinase A.
Reference(s):
– Casey et al., J Clin Invest. 2000 Sep;106(5):R31-8.
