Korean Circ J.  2007 Apr;37(4):183-186. 10.4070/kcj.2007.37.4.183.

A Case of Carney Complex: Diagnosed 11 Years after Resection of Recurrent Cardiac Myxomas

• Bae MH
• Jang SY
• Lee SH
• Lee SH
• Park Y
• Park HS
• Cho Y
• Chae SC
• Park WH
• Jun JE
• Yang DH

• KMID: 2227077
• DOI: http://doi.org/10.4070/kcj.2007.37.4.183

Abstract

Carney complex (CNC) is a rare disease characterized by myxoma, spotty skin pigmentation, and multiple neuroendo crine tumors. Here, we present a case of CNC, diagnosed 11 years after resection of recurrent cardiac myxomas. We had previously reported a 37-year-old woman in 1984 as biatrial myxomas and in 1994 as recurrent left atrial and ventricular myxomas, and at those times, she did not have any other myxomas and skin lesions. On her present admission, she had skin pigmentation and myxomatosis of breast, nostril and forearm. Upon the examination of her relatives, no characteristics of CNC were found on them. She was diagnosed as a sporadic form of CNC. If cardiac myxomas tend to be multiple and recurrent, we should consider the possibility of CNC, even other characteristics of CNC except cardiac myxomas are not present at diagnosis.

Keyword

Carney complex; Recurrent cardiac myxoma

MeSH Terms

Adult
Breast
Carney Complex*
Diagnosis
Female
Forearm
Humans
Myxoma*
Rare Diseases
Skin
Skin Pigmentation

Figure

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  Fig. 1 Echocardiographic images of cardiac myxomas. A: Transthoracic echocardiographic image of apical 4 chamber view showed biatrial myxomas in 1984. B: Recurrent Myxomas in LV and LA were noted in transesophageal echocardiography in 1994. LV: left ventricle, LA: left atrium, RA: right atrium, RV: right ventricle.

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  Fig. 2 Spotty skin pigmentations were noted on her lips and perioral skin.

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  Fig. 3 Bean sized soft masses were observed. A: Right nostril. B: Right forearm.

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  Fig. 4 Pathologic examination of her breast lesions showed fibroadenomas with abundant myxoid changes at H & E staining. H & E: hematoxylin and eosin.

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  Fig. 5 MR angiogram in 2005 showed multiple fusiform dilatations of middle and posterior cerebral arteries.

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