Primary Cardiac Neoplasms 

Updated: Mar 06, 2019
Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Eric H Yang, MD 

Overview

Practice Essentials

The most common primary cardiac tumor is the atrial myxoma, which accounts for 40-50% of all these neoplasms.[1]  The remainder of the pathological spectrum includes benign and malignant cell types. Although the overall incidence of primary cardiac neoplasms is low (0.0001-0.5% in autopsy series), these cardiac tumors provide unique diagnostic and therapeutic challenges.[2, 3, 4, 5]

Low-power photomicrograph of cardiac myxoma (hemat Low-power photomicrograph of cardiac myxoma (hematoxylin and eosin stain).
High-power photomicrograph showing the histology o High-power photomicrograph showing the histology of cardiac myxoma (hematoxylin and eosin stain). Note the dark staining polygonal cells characteristic of the tumor.

The clinical symptoms caused by cardiac tumors are generally secondary to their mass effect, local invasion, embolization, or constitutional symptoms. An intracardiac tumor mass may obstruct blood flow, compromise valve function, or induce neurological catastrophe secondary to tumor embolization. The location of the tumor determines the type of symptoms produced, which can include syncope, angina, dyspnea, edema, ascites, depression of pump function, cardiomyopathy, and pulmonary hypertension. Some tumors produce no symptoms and are found incidentally as a consequence of secondary symptoms such as stroke or evidence of peripheral embolization.

Most benign tumors can be resected completely with excellent outcomes. Consider heart transplantation in those instances in which the benign tumor is too large to resect. Resection is the treatment of choice for malignant cardiac tumors; however, long-term results are dismal, even with the addition of adjuvant therapy.[6]

Pathophysiology

Several types of tumors can arise in the heart, depending upon the tissues and structures involved. Primary neoplasms may arise from endocardium, valvular structures, primitive tissue rests, and the conduction system. Secondary or metastatic neoplasms arise from hematologic spread of the originating tissue. The physiologic derangements induced by the tumor are dependent upon the location of the mass and the tissue from which it arises.

Epidemiology

Frequency

United States

Incidence depends upon tumor cell type. Overall incidence of the disease is 0.0001-0.5% in autopsy series. Myxomas account for 40-50% of primary cardiac tumors in patients aged 30-60 years.

International

Rates of cardiac tumors in other countries parallel rates in the United States.

Mortality/Morbidity

See the list below:

  • Myxomas: The mortality rate of patients after myxoma removal does not differ significantly from that of the general population.

  • Rhabdomyoma, fibroma, fibroelastoma, hemangioma, lipoma, teratoma, and hamartoma: These are benign tumors, and mortality rates in patients with these tumors do not differ significantly from that of the general population.

  • Malignant tumors: These tumors are generally sarcomatous in nature. In spite of resections (complete or incomplete), median survival duration ranges from 10-24 months.

Race-, sex-, and age-related demographics

Primary cardiac tumors appear to occur equally in all races, unless their presence is connected directly with underlying genetic disorders that demonstrate a race predilection. The frequencies of these tumors in males and females are the same.

Cardiac tumors can occur in all age groups, but the following variations are seen:

  • Cardiac myxoma, lipomatous septal hypertrophy,[7] paraganglioma, and sarcoma tumors occur primarily in adults.

  • Rhabdomyoma, fibroma, teratoma, hamartoma, and Purkinje tumors occur primarily in children.[8]

  • Papillary fibroelastoma,[9] hemangioma, and lipoma occur in all age groups.

 

Presentation

History

Myxomas are often asymptomatic, but these tumors can produce symptoms by releasing substances that lead to inflammatory signs, including fever, tachycardia, and tachypnea. Myxomas also present as acute vascular insufficiency of the extremities from embolization. If myxomas are large, they will cause intracardiac obstruction manifested as dyspnea, syncope, or congestive heart failure.

  • Rhabdomyoma arises from the ventricles of atrioventricular (AV) valves and regress spontaneously. It presents with atrial arrhythmias and heart block. Approximately 80% of rhabdomyomas are associated with tuberous sclerosis. Although most rhabdomyomas are small, large ones may cause intracardiac obstruction.[10]

  • Fibroma,[11] hemangioma,[12] and lipoma are accompanied by signs and symptoms that are attributable to the mass effect of the tumor. Depending upon the location, these tumors may obstruct the right or left ventricular outflow, resulting in dyspnea, hepatic congestion, peripheral edema, and syncope. Arrhythmias may occur when the tumor is located at the ventricular septum.

  • Papillary fibroelastomas typically arise from the aortic or mitral valves; they may be asymptomatic or may be detected secondary to an embolic event. These typically are found incidentally on echocardiography.

  • Lipomatous septal hypertrophy and hamartoma or Purkinje tumors generally present with arrhythmias. In the former case, atrial arrhythmias and heart block are common. In the latter case, incessant ventricular tachycardia in an infant is indicative of the presence of a tumor.[13]

  • Teratoma and paraganglioma tumors directly compress the heart, causing tamponade symptoms (ie, hypotension, fatigue, dyspnea). These tumors may present with superior vena cava obstructive symptoms. Paraganglioma also may produce systemic symptoms such as hypertension, headache, flushing, and palpitations.

  • Of pheochromocytomas occurring as a cardiac tumor, 50% secrete catecholamines, producing the characteristic symptoms of hypertension, arrhythmias, anxiety, palpitations, and perspiration.[14]

  • For sarcomatous tumors, pain suggests malignancy. Dyspnea, pericardial effusions, right heart failure, and syncope are common.

Physical

Often, examination of patients with cardiac tumors yields no abnormal physical findings unless the tumor is large or produces substances that may cause secondary symptoms or findings.

  • Patients with cardiac myxoma may present with an acutely ischemic extremity and absent pulses due to embolization.

  • In a similar fashion, tumor emboli may produce neurological signs of stroke.

  • Patients with large prolapsing myxomas may have an early diagnostic sound termed the tumor plop. This sound results from the impact of the tumor against the endocardial wall. If the tumor is present in the left ventricle and compromises the mitral valve, the murmur of mitral stenosis may be heard.

  • In a similar fashion, masses producing ventricular compromise may manifest signs such as peripheral edema, hepatic enlargement, cyanosis, or clubbing.

  • Tumors that compress the heart may be manifested by the classic physical findings of cardiac tamponade, ie, jugular venous distention, muffled heart tones, and pulsus paradoxus.

  • Notably, no classic physical examination finding defines the presence of a cardiac tumor in a patient.

Causes

No distinct cause of cardiac tumors is known.

 

DDx

Differential Diagnoses

 

Workup

Laboratory Studies

Erythrocyte sedimentation rate (ESR) is a nonspecific test for which results may be elevated in the presence of a cardiac tumor.

Imaging Studies

See the list below:

  • Chest radiograph may demonstrate an enlarged cardiac silhouette or atrial enlargement.

  • MRI of the chest will demonstrate intracardiac pathology in the form of a mass.[15, 16]

  • Transesophageal echocardiography (TEE) is the test of choice.[17, 18] TEE delineates the position of the tumor and details the integrity or involvement of the septal and valvular structures. (Seventy-five percent of cardiac myxomas arise from the fossa ovalis on the left atrial side.[19] )

  • Perform fetal ultrasonography if the diagnosis is suspected in utero.

  • Cardiac catheterization is necessary only if other processes are strongly suspected.

  • Perform metaiodobenzylguanidine (MIBG) radionuclide scan for suspected paraganglioma. This scan uses a radioactive guanidine that is taken up preferentially by neuroendocrine cells.

Other Tests

ECG is ordered to detect potential arrhythmias that may be associated with tumors. These arrhythmias include atrial fibrillation, paroxysmal atrial tachycardia, and ventricular arrhythmias.

Histologic Findings

Myxomas are gelatinous in nature with rests of spindle cells (see the images below).[20]

Low-power photomicrograph of cardiac myxoma (hemat Low-power photomicrograph of cardiac myxoma (hematoxylin and eosin stain).
High-power photomicrograph showing the histology o High-power photomicrograph showing the histology of cardiac myxoma (hematoxylin and eosin stain). Note the dark staining polygonal cells characteristic of the tumor.

If DNA tetraploidy is present, suspect malignancy. Fibroelastomas are composed of endocardium, fibrous tissue, elastic fibers, and smooth muscle cells arranged in a central stock of collagen and a covering of hyperplastic endothelial cells.

Staging

No particular staging exists for cardiac tumors. The TNM nomenclature for primary cardiac tumors is not applicable due to their behavior and rarity. For metastatic disease to the heart, it is clear that M staging is appropriate.

 

Treatment

Medical Care

For those tumors presenting with cardiac arrhythmias, standard medical management of the arrhythmia is warranted until the diagnosis is established and definitive therapy undertaken. In the case of paragangliomas, treat hypertension with beta-blockade until resection is undertaken.

Surgical Care

When possible, treatment of choice for cardiac tumors is complete resection.[21, 22, 23] In patients whose tumors are determined to be malignant, adjuvant therapy (ie, chemotherapy, radiation) after resection did not affect the outcome.[6] Cardiac transplantation has been used for benign and malignant tumors.[24] Series using this therapy have been too small to use in predicting outcomes.

 

Medication

Medication Summary

For patients who present with paraganglioma, hypertension, or arrhythmias, beta-blockade is the medical treatment of choice until surgical intervention can be undertaken.

Beta-blockers

Class Summary

This category of drugs has the potential to suppress ventricular ectopy due to ischemia or excess catecholamines. In the setting of myocardial ischemia, beta-blockers have anti-arrhythmic properties and reduce myocardial oxygen demand secondary to elevations in heart rate and inotropy.

Metoprolol (Lopressor)

Selective beta1 adrenergic receptor blocker that decreases automaticity of contractions. During IV administration, carefully monitor BP, heart rate, and ECG.

Carvedilol (Coreg)

Nonselective beta-adrenoreceptor blocker with alpha-adrenergic blocking activity of equal potency. No intrinsic sympathomimetic activity has been documented.

Atenolol (Tenormin)

Selectively blocks beta1 receptors with little or no effect on beta2 receptors.

 

Follow-up

Further Outpatient Care

Annually monitor patients who have undergone resection for cardiac tumors with echocardiography in order to assess for recurrences. With the more aggressive malignant tumors, follow up every 3-6 months.

Complications

Complications encountered after resection of cardiac tumors can include valvular insufficiency, complete heart block requiring pacemaker insertion, atrial and ventricular arrhythmias, and stroke.

Prognosis

Prognosis for benign tumors is excellent, matching that of the healthy population.[25]

Malignant tumors carry a dismal prognosis even in the face of complete resection. An occasional sarcoma can be resected completely, resulting in long-term survival.