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Case 375 - Discussion

Uploaded: 2009-10-30, Updated: 2009-11-02

 

Cardiac Myxoma

CLINICAL FEATURES

  • Most common primary tumor of heart (~40%), mostly in left atrium.

  • Mean age 50 years.

  • Symptoms: ball-valve obstruction, emboli, fever/malaise,

  • Sporadic or

  • 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).

GROSS FINDINGS

  • Soft, pale, lobulated, polypoid, sessile or pedunculated

MICROSCOPIC FINDINGS

  • Tumor cells arranged in cords, nests, rings or poorly formed glands, often surrounding blood vessels.

  • Stellate or globular myxoma cells, abundant eosinophilic cytoplasm, indistinct cell borders, oval nucleus with open chromatin and indistinct nuclei.

  • Abundant myxoid background.

  • Often inflammation, hemorrhage.

  • Other features: fibrosis, calcification, Gamna-Gandy bodies, ossification, extramedullary hematopoiesis.

IMMUNOPHENOTYPE

  • Positive: CD31, CD34, calretinin (strong, diffuse, cytoplasmic and nuclear), S100, vimentin, cytokeratin+ in glandular components; variable Factor VIII+.

  • Negative: CD68, cytokeratin (except for glandular elements).

TREATMENT AND PROGNOSIS

  • Benign tumor, no or rare recurrence after excision.

REFERENCES: Enzinger and Weiss's Soft Tissue Tumors, 5th Edition. By Drs. Sharon Weiss and John Goldblum. 2007