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Presented by George Netto, M.D. and prepared by Joseph J. Maleszewski, M.D.
Case 2: A 37-year-old woman was found to have a left atrial cardiac mass.
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Question 1 of 1
1. Question
Week 355: Case 2
A 37-year-old woman was found to have a left atrial cardiac mass during a work-up for a recent cerebrovascular accident.images/jjm062408/Case 2/a.jpg
images/jjm062408/Case 2/b.jpg
images/jjm062408/Case 2/c.jpg
images/jjm062408/Case 2/d.jpgCorrect
Answer: Cardiac myxoma
Histology: Cardiac myxomas are composed of stellate/polygonal mesenchymal cells set in a mucopolysaccharide-rich background matrix. Hemosiderin depositions in the form of Gamna Gandy bodies composed of iron encrusted fibers, glandular epithelial structures and extramedulary hematopoiesis are also rarely encountered in CM.
Discussion: Cardiac Myxoma (CM) and papillary fibroelastomas are the two most common primary benign cardiac tumors. Located most commonly in the left atrium, CM are thought to arise from pluripotent mesenchymal cells. The latter may account for unusual bone and other mesenchymal elements found in rare CM lesions.
Although the majority of CM are sporadic lesions, they can be familial in nature. The latter include the occurrence of CM as part of Carney’s complex. Primary pigmented nodular adrenocortical disease (PPNAD), large cell calcifying testicular Sertoli Tumors, myxomatous fibroadenoma of breast, skin myxomas and oral pigmentations are other components of Carney’s complex.
References:
1- Vaideeswar P, Butany JW. Benign cardiac tumors of the pluripotent mesenchyme. Semin Diagn Pathol. 2008 Feb;25(1):20-8. Arch Pathol Lab Med 2007; 131(4):599-603
2- Lee B, Sir JJ, Park SW, Kim SB, Nah JC, Kang YK, Lee HK, Kim YI, Cho WH, Choi SK. Right-sided myxomas with extramedullary hematopoiesis and ossification in Carney
complex.Reference(s):
– Int J Cardiol. 2008Incorrect
Answer: Cardiac myxoma
Histology: Cardiac myxomas are composed of stellate/polygonal mesenchymal cells set in a mucopolysaccharide-rich background matrix. Hemosiderin depositions in the form of Gamna Gandy bodies composed of iron encrusted fibers, glandular epithelial structures and extramedulary hematopoiesis are also rarely encountered in CM.
Discussion: Cardiac Myxoma (CM) and papillary fibroelastomas are the two most common primary benign cardiac tumors. Located most commonly in the left atrium, CM are thought to arise from pluripotent mesenchymal cells. The latter may account for unusual bone and other mesenchymal elements found in rare CM lesions.
Although the majority of CM are sporadic lesions, they can be familial in nature. The latter include the occurrence of CM as part of Carney’s complex. Primary pigmented nodular adrenocortical disease (PPNAD), large cell calcifying testicular Sertoli Tumors, myxomatous fibroadenoma of breast, skin myxomas and oral pigmentations are other components of Carney’s complex.
References:
1- Vaideeswar P, Butany JW. Benign cardiac tumors of the pluripotent mesenchyme. Semin Diagn Pathol. 2008 Feb;25(1):20-8. Arch Pathol Lab Med 2007; 131(4):599-603
2- Lee B, Sir JJ, Park SW, Kim SB, Nah JC, Kang YK, Lee HK, Kim YI, Cho WH, Choi SK. Right-sided myxomas with extramedullary hematopoiesis and ossification in Carney
complex.Reference(s):
– Int J Cardiol. 2008