["Myxoma syndrome"--a "benign" disease with "malignant" course]

We are reporting on a 36 year-old woman who presented with recurrent cardiac myxomas over a period of nine years. Two of the tumors typically originated in the left atrium and one in the right atrium. Tumor embolization was the presenting symptom twice, leading to reversible cerebral ischemia and minor pulmonary embolism, respectively. The third tumor remained asymptomatic and was detected during routine echocardiographic examination. Based on a positive family history of cardiac tumors, a facially pronounced hyperpigmentation of the skin and the presence of a thyroid adenoma, the diagnosis of a "myxoma syndrome" was established. Patients with "myxoma syndrome" are generally younger than their counterparts with "sporadic myxoma" (mean age at diagnosis 25 vs. 56 years) and have a high frequency of unusual skin freckling (68%). Familial clustering of cardiac myxomas is also frequent (25%). The tumors may be located in any of the cardiac chambers (87% in the atrias, 13% in the ventricles, 50% at multiple sites simultaneously) and have relatively high (18%) 5-year recurrence rate after surgical excision. Since the clinical signs of cardiac tumors are non-specific, diagnosis essentially relies on cardiac imaging by echocardiography, computer tomography, or angiography. The superiority of transesophageal echocardiography is emphasized in this report.