Most people don’t realize that there can be tumors of the heart. In fact, tumors of the heart are not uncommon.
Heart tumors can be either benign or malignant. Approximately 70% of primary heart tumors are benign and 30% are malignant. Unfortunately, the heart can also be the site of metastatic tumors from other sites.
Benign Heart Tumors (70%)
- Myxoma
- Lipoma
- Papillary Fibroelastoma
- Rhabdomyoma
- Fibroma
- Hemangioma
- Teratoma
- Mesothelioma of AV Node
- Granular Cell Tumor
- Neurofibroma
- Lymphangioma
Malignant Heart Tumors (30%)
- Angiosarcoma
- Rhabdomyosarcoma
- Mesothelioma
- Fibrosarcoma
- Malignant Lymphoma
- Extraskeletal Osteosarcoma
- Neurogenic Sarcoma
- Malignant Teratoma
- Thymoma
- Leiomyosarcoma
- Liposarcoma
- Synovial Sarcoma
Atrial Myxoma
Atrial myxomas are tumors that occur within the chambers of the heart, usually the left atrium. They account for 29% of all heart tumors. Although generally considered benign, there are reports of metastasis to other parts of the body.
80-90% of myxomas occur in the left atrium. They are usually 5-6 cm in size, but can range from 1-15 cm. Left atrial myxomas are usually attached to the septum of the heart separating the left and right atrium.
Their appearance is typically that of a gelatinous ball projecting into the left atrial chamber, with soft projections, sort of like a small jellyfish! When removing one of these tumors, it is important not to have any of this soft material break off and drop into the heart.
Since these tumors are usually attached the the septal lining, a combination of a left atrial and right atrial incision is often required. A portion of the septum between the left and right atrium is removed with the tumor and the septum is patched with a piece of the patient’s own pericardium or a piece of processed bovine (cow) pericardium.
Case Report 1
Below is a typical case of a left atrial myxoma. The tumor was relatively small and was able to be removed with a single incision in the patient’s left atrium.
TEE (Trans-Esophageal Echocardiogram) showing a left atrial myxoma
Below is a black and white photograph of a pathologic specimen of the above tumor. Note the jelly-like appearance of the cut tumor.
Pathologic Specimen of Tumor
Case Report 2
This case shows an extraordinarily large left atrial tumor. An incision was made in both the left and right atrium and a portion of the atrial septum was removed in order to safely remove the tumor.
TEE (Trans-Esophageal Echocardiogram) showing a giant left atrial myxoma
Below is a color photograph of a pathologic specimen of the tumor. This tumor was surprisingly solid.
Solid Left Atrial Myxoma Tumor
Here’s a really cool video showing my removing a large left atrial myxoma. These are relatively rare operations, so to see a real video is special.
Cardiac Papillary Fibroelastoma
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.
