Carney Complex Treatment & Management
- Author: Craig T Basson, MD, PhD; Chief Editor: Richard A Lange, MD more...
Medical Care
- Evaluation usually can be performed in an outpatient setting.
- No specific drug is useful in the management of myxomas. Anticoagulation is not indicated in patients with myxomas and does not reduce the risk of emboli. Emboli arising from intracardiac myxomas are due to fragments of the tumor that break off and embolize.
- Medical treatment of endocrine overactivity may be required.
Surgical Care
- Mass lesions usually require biopsy/resection to provide a histopathologic diagnosis.
- Surgery is necessary to resect intracardiac myxomas and to prevent embolic stroke or valvular obstruction. Recognize that even in the presence of resections with adequate surgical margins, intracardiac myxomas may recur at sites distant from the initial operative site. These recurrences do not reflect a failure of the initial surgical treatment but, rather, a genetic predisposition to recurrent tumor formation. Although recurrence of cardiac myxomas (and similarly extracardiac myxomas) is a feature of Carney complex, it is not a feature of typical nonsyndromic sporadic atrial myxomas.
- Resect extracardiac myxomas and nonmyxomatous benign lesions if they produce symptoms by local extension of the space-occupying lesion.
- Rare malignant tumors require resection and may require other adjunctive therapy and referral to an oncologist.
Consultations
- Cardiologist
- Geneticist
- Cardiothoracic surgeon
- Endocrinologist
- Oncologist
Diet
No special diet is required.
Activity
In the absence of heart failure or persistent cardiac sequelae, no activity limitation is required.
Basson CT, MacRae CA, Korf B, Merliss A. Genetic heterogeneity of familial atrial myxoma syndromes (Carney complex). Am J Cardiol. Apr 1 1997;79(7):994-5. [Medline].
Stratakis CA, Carney JA, Lin JP, et al. Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2. J Clin Invest. Feb 1 1996;97(3):699-705. [Medline].
Casey M, Mah C, Merliss AD, et al. Identification of a novel genetic locus for familial cardiac myxomas and Carney complex. Circulation. Dec 8 1998;98(23):2560-6. [Medline].
Casey M, Vaughan CJ, He J, et al. Mutations in the protein kinase A R1alpha regulatory subunit cause familial cardiac myxomas and Carney complex. J Clin Invest. Sep 2000;106(5):R31-8. [Medline].
Veugelers M, Bressan M, McDermott DA, Weremowicz S, Morton CC, Mabry CC, et al. Mutation of perinatal myosin heavy chain associated with a Carney complex variant. N Engl J Med. Jul 29 2004;351(5):460-9. [Medline].
Reynen K. Cardiac myxomas. N Engl J Med. Dec 14 1995;333(24):1610-7. [Medline].
Goldstein MM, Casey M, Carney JA, Basson CT. Molecular genetic diagnosis of the familial myxoma syndrome (Carney complex). Am J Med Genet. Sep 3 1999;86(1):62-5. [Medline].
Carney JA, Gordon H, Carpenter PC, et al. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore). Jul 1985;64(4):270-83. [Medline].
Kanda T, Umeyama S, Sasaki A, et al. Interleukin-6 and cardiac myxoma. Am J Cardiol. Nov 1 1994;74(9):965-7. [Medline].
Veugelers M, Wilkes D, Burton K, et al. Comparative PRKAR1A genotype-phenotype analyses in humans with Carney complex and prkar1a haploinsufficient mice. Proc Natl Acad Sci U S A. Sep 28 2004;101(39):14222-7.
Print
