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Cardiac
Myxoma |
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CLINICAL FEATURES |
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Most common primary tumor of heart (~40%),
mostly in left atrium.
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Mean age 50 years.
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Symptoms: ball-valve obstruction, emboli,
fever/malaise,
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Sporadic or
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10% familial, associated with Carney
syndrome: autosomal dominant with multiple cardiac and cutaneous
myxomas, spotty skin pigmentation, endocrine overactivity,
schwannomas, epithelioid blue nevus. Younger, mean 24 years;
usually men (66% vs. 24% in non-familial cases), multicentric
(33%); caused by mutations in protein kinase A regulatory
subunit 1 alpha (PRKAR1A).
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GROSS FINDINGS |
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Soft,
pale, lobulated, polypoid, sessile or pedunculated
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MICROSCOPIC FINDINGS |
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Tumor cells arranged in cords, nests, rings
or poorly formed glands, often surrounding blood vessels.
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Stellate or globular myxoma cells, abundant
eosinophilic cytoplasm, indistinct cell borders, oval nucleus
with open chromatin and indistinct nuclei.
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Abundant myxoid background.
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Often inflammation, hemorrhage.
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Other features: fibrosis, calcification,
Gamna-Gandy bodies, ossification, extramedullary hematopoiesis.
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IMMUNOPHENOTYPE |
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Positive: CD31, CD34, calretinin (strong,
diffuse, cytoplasmic and nuclear), S100, vimentin, cytokeratin+
in glandular components; variable Factor VIII+.
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Negative: CD68, cytokeratin (except for
glandular elements).
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TREATMENT AND PROGNOSIS |
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REFERENCES: Enzinger and Weiss's Soft Tissue
Tumors, 5th Edition. By Drs. Sharon Weiss and John Goldblum.
2007 |