Approach Considerations
No specific drug is useful in the management of myxomas in Carney complex, with resection being the treatment of choice for cardiac myxomas. Anticoagulation also is not indicated in patients with myxomas and does not reduce the risk of emboli (which, in cardiac myxomas, are actually fragments of the tumor that have broken off). [4]
Medical care in Carney complex is restricted to the treatment of endocrine overactivity, which is commonly present. Antibiotic prophylaxis is required if valvular insufficiency exists.
In the absence of heart failure or persistent cardiac sequelae, no activity limitation is required in Carney complex.
Consultations
Consultations with the following specialists may be indicated in Carney complex:
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Cardiologist
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Geneticist
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Cardiothoracic surgeon
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Endocrinologist
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Oncologist
Monitoring
Patients who have Carney complex should be monitored annually with echocardiography to detect the occurrence of new cardiac myxomas. The relapse rate for these myxomas after removal is high. [36]
Annual history and physical examination also are required, to detect the development of extracardiac myxomas and other tumors, as well as the signs and symptoms of endocrine dysfunction.
Tumor Resection
As previously stated, mass lesions in Carney complex usually require biopsy/resection to provide a histopathologic diagnosis.
Surgery is necessary to resect cardiac myxomas and to prevent embolic stroke or valvular obstruction. [4] Recognize that even in the presence of resections with adequate surgical margins, cardiac myxomas may recur at locations distant from the initial operative site. These recurrences do not reflect a failure of the initial surgical treatment but instead reveal 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.
Extracardiac myxomas and nonmyxomatous benign lesions should be resected if they produce symptoms via local extension. Large or symptomatic skin myxomas also can be excised.
Rare malignant tumors require resection, as well as, possibly, adjunctive therapy and referral to an oncologist.
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Transthoracic echocardiogram of a left atrial myxoma in an individual with Carney complex. A 42-year-old woman with a history of spotty pigmentation on the face and cutaneous myxomas presented for annual surveillance echocardiography. Findings from previous echocardiograms were normal. Echocardiography now revealed a 1.0 X 1.3-cm mass (arrow) in the left atrium (LA) arising from the interatrial septum above the mitral valve. No prolapse was seen into the left ventricle (LV). Histopathology upon surgical excision demonstrated that the lesion was a myxoma.
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A pedunculated, flesh-colored cutaneous myxoma that is 1.5 cm in diameter on trunk. Courtesy of Dermatology, NYU, and Ann Stoecker, medical photographer.
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Polypoid neoplasm of fibrillary collagen and uniform stellate cells within abundant connective tissue mucin. Note telangiectasia and ramification of tumor as strands through a myxoid dermis (hematoxylin-eosin). Courtesy of Dermatology, NYU School of Medicine; photography by Anca Croitoru, MD, and Scott Sanders, MD.
