INTRODUCTION	Section 2 of 11
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures Bibliography

Background: The most common primary cardiac tumor is the atrial myxoma, which accounts for 40-50% of all these neoplasms. The remainder of the pathological spectrum includes benign and malignant cell types. Although the overall incidence of the disease is low (0.0001-0.5% in autopsy series), it provides unique diagnostic and therapeutic challenges.

The clinical symptoms caused by cardiac tumors generally are 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, eg, stroke or evidence of peripheral embolization.

Most benign tumors can be resected completely with excellent outcomes. Consider heart transplantation in those instances when 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.

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.

Frequency:

• In the US: 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.

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

Mortality/Morbidity:

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

Race: 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.

Sex: The frequencies of these tumors in males and females are the same.

Age: Cardiac tumors can occur in all age groups.

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

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

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

	CLINICAL	Section 3 of 11
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures Bibliography

History: Myxomas often are 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.

• Fibroma, hemangioma, 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.

• 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.

• 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.