A cardiac tumor is an abnormal growth of tissue in the heart, and may be classified as either cancerous (malignant) or non-cancerous (benign). Cardiac tumors are further classified as either primary (originating within the heart itself) or secondary (spread from a primary tumor in a different part of the body). Primary cardiac tumors are extremely rare, occurring in approximately 1 in 3000 individuals. Seventy-five percent of primary cardiac tumors are benign. Secondary cardiac tumors are 20-30 times more frequent than primary cardiac tumors and most commonly arise in patients with lung cancer, breast cancer, melanoma, renal cell cancer, or lymphoma.
What are the symptoms of a cardiac tumor?
Many patients with a cardiac tumor are asymptomatic (have no symptoms at all). For those patients that do have symptoms they are typically non-specific and similar to many other more common cardiovascular conditions. Symptom presentation depends primarily upon tumor location, size, growth rate, and friability (tendency to break off and travel in the blood stream).
Examples of clinical presentations include:
- Heart failure (shortness of breath, leg swelling, inability to lie flat)
- Arrhythmia (irregular heart beat)
- Chest pain
- Stroke (slurred speech, weakness, vision loss)
- Pericardial effusion (fluid/blood/tumor within the sac that surrounds the heart)
- Constitutional symptoms (fever, weight loss, elevated inflammatory markers, anemia)
How is a cardiac tumor detected?
Cardiac tumors are most commonly diagnosed by echocardiogram (cardiac ultrasound). Further non-invasive imaging with computed tomography (CT scan), magnetic resonance imaging (MRI), or positron emission tomography (PET scan) may help to better characterize/diagnose a cardiac tumor.
What are the different types of primary cardiac tumors?
The most common types of benign heart tumors are:
- Myxoma – most common primary cardiac tumor. Account for approximately 50% of primary cardiac tumors.
- Papillary fibroelastoma – most common cardiac tumor to affect the cardiac valves. Mean age at diagnosis is 60 years old. These tumors are associated with embolization (breaking off and traveling in the blood stream) resulting in stroke or less commonly heart attack.
- Rhabdomyoma – most common cardiac tumor in infants and children. Typically multiple and originating from the ventricular wall. Associated Tuberous Sclerosis is seen in 1/3 of patients.
- Fibroma – commonly occur in infants and children. Typically located within the ventricular wall. Associated with arrhythmia (irregular heart beat) and risk for sudden cardiac death.
- Lipoma – tumor made up of fat cells. Diagnosis can be made non-invasively by cardiac MRI.
- Hemangioma – tumor made of abnormal blood vessels. Diagnosis can be made either invasively on coronary angiography or non-invasively on cardiac CT or MRI.
Nearly all primary malignant cardiac tumors are sarcomas, and the most frequent one is angiosarcoma. Angiosarcoma is a very aggressive form of cancer made up of abnormal blood vessels. It characteristically originates from the right atrium. The clinical course is rapid with a very high incidence of spread throughout the body (metastasis). Once the cancer has metastasized the prognosis is poor.
Myxoma is a non-cancerous (benign) tumor of the heart. In fact, it is the most common primary heart tumor. A primary heart tumor is one that originates within the heart as opposed to another organ or tissue. Approximately 75% of myxomas are located within the left atrium. Typically, myxomas grow off of the inter-atrial septum (wall that separates the two atria). Myxomas tend to be solitary (occur as a single mass).
The cause of myxoma is unknown. Myxomas are more common in females than in males. Approximately 10% of myxomas are thought to be genetic (passed down through families). These are called familial myxomas and are more likely to be numerous and occur in younger patients.
Many patients with a myxoma do not have any symptoms. For those patients that do have symptoms they may have difficulty breathing when lying flat, chest pain or tightness, coughing, dizziness, fainting, fevers, or flu-like illness.
The diagnosis of myxoma is most commonly made by echocardiography (cardiac ultrasound). Additionally, computed tomography (CT scan) or magnetic resonance imaging (MRI) may confirm the diagnosis.
The only treatment of a myxoma is surgical excision. This needs to be performed by a highly skilled cardiac surgeon because incomplete removal can result in recurrence of the tumor. Once a patient is diagnosed with a myxoma, surgical excision is usually recommended to prevent complications. These include arrhythmias (irregular heart rhythm), heart failure, embolism (breaking off of tumor cells which then travel within the bloodstream) which can cause stroke or blockage of blood flow to other organs.
Periodic echocardiographic evaluation is recommended for patients with a history of surgical excision of a myxoma.
Treatment begins with a thorough diagnostic workup to fully characterize the tumor in order to understand the type of tumor, its size and anatomic location, as well as any spread outside of the heart. This typically requires blood work and non-invasive cardiac imaging. The importance of an accurate assessment of a cardiac tumor prior to initiation of therapy cannot be overstated. In collaboration with the Division of Radiology at NYP/Columbia, our Cardiac Tumor Program offers the most advanced non-invasive imaging capabilities available anywhere in the world.
Following characterization of a cardiac tumor, our multi-disciplinary care team, which includes cardiologists, radiologists, oncologists, and cardiac surgeons, sit down together to develop an individualized treatment plan in order to achieve the optimal outcome.
Treatment options are dependent upon several factors including type of tumor, tumor size and location, presence or absence of systemic metastases and the patient's overall health status.
In general, patients with a primary cardiac tumor require surgical resection. However, depending upon the tumor size and location, complete removal may not be possible with standard surgical techniques. Our surgeons are world-renowned and have extensive experience in high-risk and complex cardiac procedures including heart reconstructive surgery, heart replacement therapy with left ventricular assist device (LVAD) or Syncardia Total Artificial Heart (TAH), and heart transplantation.
Following excision of a malignant tumor, patients may require chemotherapy and/or radiation therapy to prevent recurrence of the tumor.
If you have heart disease and need help, we’re here for you. To get started today, call (212) 342-3820 or use our appointment request form.