Cardiac papillary fibroelastoma is the most common tumor of heart valves. It is found most often on the aortic valve. Although benign (non-cancerous), these tumors are increasingly being recognized as a source of systemic embolization that can lead to transient ischemic attacks (TIA), ischemic stroke, and heart attacks.
The tumor pictured below is a papillary fibroelastoma of the aortic valve, found incidentally in a patient who was being evaluated for mitral valve surgery.
The appearance below is classically described as a “sea anemone” with a central stalk and frond-like arms projecting outwardly. Looking at the appearance of these unique tumors, it’s easy to understand how the tissue particles could embolize during the movement of the heart valves with each heartbeat.
Histologically, the composition of these tumors are made up of collagen and elastin, with very little vascularure. The outer layer is comprised of endothelium, an intermediate layer of connective tissue, and a core of fibrin.
Clinically, a small asymptomatic tumor may be monitored, but surgical excision is the standard recommended treatment for larger, mobile tumors, unless there is a contraindication to surgery. The heart valve itself can usually be spared and/or repaired; however, if there is significant underlying damage to the valve, it may warrant valve replacement at the time of tumor removal.
In this case example, the patient underwent the planned mitral valve operation via a sternotomy and a left atrial incision for the mitral valve portion of the operation. A second incision was then made in the aorta to remove the aortic valve tumor and successfully repair the aortic valve. The patient had no further aortic or mitral valve insufficiency. The tumor was completely excised.
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