Multiple and recurrent cardiac myxomas should raise suspicion for Carney complex, even if other characteristic features are absent at initial presentation.
Recurrent myxomas may signal Carney complex despite delayed signs; extends phenotypic spectrum but leaves screening protocols open.
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. (Korean Circulation J 2007;37:183-186)
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Bae et al. (2007) studied this question.
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