Primary Cardiac Neoplasms Clinical Presentation

Updated: Oct 14, 2015
  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Eric H Yang, MD  more...
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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. [9]
  • Fibroma, [10] hemangioma, [11] 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. [12]
  • 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. [13]
  • For sarcomatous tumors, pain suggests malignancy. Dyspnea, pericardial effusions, right heart failure, and syncope are common.
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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.
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Causes

No distinct cause of cardiac tumors is known.

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