Surgical treatment of left-atrial myxoma in Carney's complex

Although rare, cardiac myxomas are the most frequently encountered primary neoplasms of the heart. Cardiac myxomas are typically sporadic, benign, non-recurrent tumors, and they are usually seen in the left atrium. Patients who have myxoma-associated spotty pigmented skin lesions, endocrine neoplasms with overactivity, and noncardiac myxomatous tumors are said to have Carney's complex. This report presents a 21-year-old woman with Carney's complex. She was operated for tetralogy of Fallot and total correction was performed at another center when she was three years old. Two years ago, she was operated on for a primary pigmented nodular adrenocortical tumor and bilateral adrenalectomy was performed. Her present admission to our clinic was for left-atrial myxoma with associated symptoms. Patients with Carney's complex have distinctive clinical features which separate them from the larger group of patients with the more common sporadic myxoma. The most important distinction is that the myxoma syndrome appears to be a multisystem disease.