Familial Cushing's Syndrome (Carney Complex)
- 17 May 1990
- journal article
- letter
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 322 (20) , 1469-1470
- https://doi.org/10.1056/nejm199005173222015
Abstract
We commend Dr. Young et al. (Dec. 14 issue)* for continuing to educate the medical community about the Carney complex, a rare autosomal dominant disorder characterized by mesenchymal tumors, spotty skin pigmentation, functional endocrine overactivity, and peripheral-nerve tumors. We recently cared for a 26-year-old black woman with a history of Cushing's syndrome. Workup revealed a left adrenal mass presumed to be an adenoma and, in addition, an incidental mass arising from the greater curvature of the stomach. The patient underwent successful adrenal surgery and removal of a pigmented psammomatous schwannoma on the greater curvature of the stomach. Because these findings were recognized as components of the Carney complex, two-dimensional echocardiography was performed, revealing two intracardiac masses (involving the free wall of the right atrium and a papillary muscle of the left ventricle). Surgical resection identified them as cardiac myxomas.Keywords
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