Carney Complex: A Rare Case of Multicentric Cardiac Myxoma Associated with Endocrinopathy

Abstract

Carney complex is a rare autosomal dominant disorder characterized by multiple tumors, including cardiac and extracardiac myxomas, skin lesions, and various endocrine disorders. We are reporting a 21-year-old female patient with past surgical history significant for excision of a cutaneous myxoma who presented with multicentric cardiac myxomas involving the four cardiac chambers. She also presented with endocrinal disorders in the form of an enlarged right lobe of the thyroid, hyperthyroid state, and an incidentally noted adrenal cyst; hence, she was diagnosed with carney complex syndrome.

Multimodality Cardiac Imaging Enhances Diagnosis and Management of Recurrent Atrial Myxomas in Carney Complex

“… 1 They can recur in the same 5 or different 6 sites and present in one or all four chambers. 7 Prior literature reports that nearly 80% of myxomas are localized to the left atrium, 7%-20% in the right atrium, 2.5%-6% in the right ventricle, 8% in the left ventricle, 2.5% in two or more locations, and <2.5% in both atria of these same patients. 8 Cardiac myxomas seen in sporadic cases may recur in 3% of patients.…”

Carney Complex: “The Complex of Cardiac Myxoma”

“…Carney triad encompasses three types of tumors: a gastric stromal sarcoma, functioning extra-adrenal paragangliomas, and pulmonary chondromas [1]. Cardiac myxomas may cause strokes due to embolism in addition to heart failure [2]. It is therefore important to screen regularly at least once a year by echocardiogram.…”

“…About 500 patients have been registered by the NIH-Mayo Clinic (USA) and the Cochin center (France) [1]. Cumulative reports from these centers, plus information from the Cornell center in New York, indicate that there are about 160 index cases of CNC presently known since the disorder was first described in the medical literature in 1985 [2].…”

“…Cardiac myxomas can develop in any cardiac chamber and may be multiple. They comprise main feature of Carney Complex in 30-60% of cases and may be responsible for the high rate (16%) of sudden death historically reported in CNC families [2].…”