The authors report the case of a rare clinical syndrome which has recently been called Carney's complex or "myxomas, spotty pigmentation and endocrine overactivity". Three components of this complex are described: cutaneous myxomas, Cushing's syndrome of unpredictable evolution treated by bilateral adrenalectomy for multiple adrenal adenoma and left atrial myxoma which recurred twice. The authors review the literature and discuss the practical implications of this new syndrome which may be familial. The role of echocardiography, the key investigation for the detection of the myxoma and follow-up of these patients who have a high risk of recurrence, is underlined.

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