Abstract
Carney complex (CNC) is a disorder characterized by skin pigmentary abnormalities and benign cardiac,endocrine, skin and neuronal tumors. Areas of unusual lentigines are the most common presenting feature of CNC usually around the lips, eyes or genitalia increase in number at puberty. Cardiac myxomas may occur in any or all cardiac chambers, and leading to intracardiac obstruction of blood flow, embolic phenomena, and/or heart failure. Carney complex is a rare autosomal dominant disease. The complex has been mapped to 2p16 (CNC2) and 17q22-24 (CNC1), and mutations in the tumor suppressor gene protein kinase A regulatory subunit 1-alpha, PRKAR1A, are causative [1].
Highlights
Carney complex (CNC) is a disorder characterized by skin pigmentary abnormalities and benign cardiac,endocrine, skin and neuronal tumors
An echocardiography revealed an obstructive tumor, 5-6 centimeters in diameter, in the left atrium, which was surgically removed.The lentigines and the myxoma led to the suggestion of Carney Complex
To our knowledge, this is the first case of combined Carney Complex and cryoglobulinemia
Summary
Carney complex (CNC) is a disorder characterized by skin pigmentary abnormalities and benign cardiac,endocrine, skin and neuronal tumors. Areas of unusual lentigines are the most common presenting feature of CNC usually around the lips, eyes or genitalia increase in number at puberty. Cardiac myxomas may occur in any or all cardiac chambers, and leading to intracardiac obstruction of blood flow, embolic phenomena, and/or heart failure. Carney complex is a rare autosomal dominant disease. The complex has been mapped to 2p16 (CNC2) and 17q22-24 (CNC1), and mutations in the tumor suppressor gene protein kinase A regulatory subunit 1-alpha, PRKAR1A, are causative [1]
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