Familial Recurrent Atrial Myxoma: Carney's Complex
Article first published online: 6 FEB 2011
© 2011 Wiley Periodicals, Inc.
Volume 34, Issue 2, pages 83–86, February 2011
How to Cite
Nagesh Shetty Roy, A., Radin, M., Sarabi, D. and Shaoulian, E. (2011), Familial Recurrent Atrial Myxoma: Carney's Complex. Clin Cardiol, 34: 83–86. doi: 10.1002/clc.20845
- Issue published online: 4 FEB 2011
- Article first published online: 6 FEB 2011
- Manuscript Accepted: 7 SEP 2010
- Manuscript Received: 3 JUL 2010
We report on a family of 4 members, all of whom have had multifocal, recurrent atrial myxomas associated with skin pigmentation, melanotic schwannomas, mucocutaneous myxomas, and tumors of the ovary and pituitary, adrenal, and thyroid glands. Immunochemistry of the myxoma cells is positive for calretinin, confirming their neuroendocrine origin. Genetic studies confirmed mutations in the gene coding protein kinase A, regulatory subunit 1-α (PRKAR1α). This is Carney's complex, characterized by multiple, mucocutaneous myxomas; pigmented lesions over the lips, conjunctiva, and genitalia; adenomas of the breast and thyroid; schwannomas; and endocrinal abnormalities including Cushing syndrome and acromegaly. Members of the family require vigorous screening, including urinary free cortisol, plasma transforming growth factor-β1 and thyrotropin-releasing hormone, testicular ultrasound, routine echocardiographic screening, searches for cardiac and mucocutaneous myxomas in multiple locations, and genetic studies for the PRKAR1α gene sequence. Copyright © 2011 Wiley Periodicals, Inc.
The authors have no funding, financial relationships, or conflicts of interest to disclose.