Carney Complex: “The Complex of Cardiac Myxoma

Abstract

The Carney complex (CNC) is a rare dominantly inheritedsyndrome characterized by spotty skin pigmentation, endocrine overactivity and myxomas. About 500 patients have been registered by the NIH-Mayo Clinic (USA) and the Cochin center (France)[1]. Cumulative reports from these centers, plus information fromthe Cornell center in New York, indicate that there are about 160index cases of CNC presently known since the disorder was firstdescribed in the medical literature in 1985 [2]. It affects males and females in equal numbers and can present at any age with the mean age at diagnosis being 20. Cardiac myxomascan develop in any cardiac chamber and may be multiple. Theycomprise main feature of Carney Complex in 30-60% of casesand may be responsible for the high rate (16%) of sudden deathhistorically reported in CNC families [2].Carney Complex is characterized by spotty skin pigmentation,cardiac (heart) myxomas (tumors composed of mucous connectivetissue), skin myxomas, endocrine tumors or over-activity, andschwannomas.