Abstract
Carney complex is a rare syndrome caused by a genetic mutation leading to multiple endocrine abnormalities and a variety of tumors. Here, we report a case of Carney complex diagnosed due to recurrent multiple myxomas in the right atrium of a patient 16 years after the resection of the primary left atrial myxoma. Surgical excision was performed for the multiple recurrent right atrial tumors under cardiopulmonary bypass. The patient remained complication-free after surgery and was discharged on the 14th day. He was scheduled to continue echocardiographic follow-up and periodic systemic review by an endocrinologist. This case emphasizes the fact that if cardiac myxomas tend to be multiple and recurrent at a relatively young age, the possibility of Carney complex should be considered, even in the absence of any other related feature other than cardiac tumors.
Highlights
CaseCarney complex (CNC) is a rare syndrome of multiple endocrine neoplasia characterized by characteristic pigmented lesions on the skin and mucosal surfaces, cardiac and noncardiac mucinous tumors, and multiple endocrine tumors [1, 2]
57% of deaths associated with CaseCarney complex (CNC) are cardiac-related deaths, including those due to cardiac tumors
We report a case of CNC diagnosed 16 years after left atrial myxoma resection, discovered due to recurrent multiple myxomas in the right atrium
Summary
Carney complex (CNC) is a rare syndrome of multiple endocrine neoplasia characterized by characteristic pigmented lesions on the skin and mucosal surfaces, cardiac and noncardiac mucinous tumors, and multiple endocrine tumors [1, 2]. We report a case of CNC diagnosed 16 years after left atrial myxoma resection, discovered due to recurrent multiple myxomas in the right atrium. The patient had previously undergone a left atrial myxomectomy at the age of 25 years He was diagnosed after a systemic examination due to stroke at a young age. At the age of 41 years, the first postoperative neoplastic lesion in the right atrium was noted, and the patient was referred to our hospital. Physical examination performed following admission to the hospital revealed pigmented patches on the face and lips. His blood pressure and pulse rate were normal, and he had no subjective symptoms.
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