Pediatric Cardiac Tumors Clinical Presentation

Updated: Jan 04, 2016
  • Author: Edwin Rodriguez-Cruz, MD; Chief Editor: Syamasundar Rao Patnana, MD  more...
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Presentation

History

Diagnosis is a challenge for any physician because cardiac tumors have no typical presentation. Typically, patients are asymptomatic or present with nonspecific signs and symptoms. Some authors call heart neoplasms the great masqueraders. Certain symptoms, including irritability, shortness of breath, anorexia, tiredness, or palpitations, may raise suspicion of a neoplastic process.

Note the following:

  • Initial symptoms in infants may include irritability, periodic episodes of pallor, fever, tachypnea, tachycardia, anorexia, and failure to thrive. [5]

  • Older children and adolescents may present with similar symptoms and may complain of dyspnea on exertion, dizziness, general malaise, syncope, hemoptysis, and shock or experience sudden death.

  • For presenting symptoms of specific types of cardiac tumors, see Background.

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Physical

Clinical presentation and physical findings relate to location of the tumor. Arrhythmias, heart failure, fever, pericardial effusion, and new or louder heart murmurs are a few of the findings for all these growths (see Background).

Note the following:

  • Right-sided tumors (see image below): Heart failure, edema, jugular venous distention, ascites, shortness of breath, right-sided third and/or fourth heart sounds, cor pulmonale, pericardial effusions, hepatomegaly, vena cava syndrome, and pulmonary embolism are associated with right-sided tumors.

    Echocardiographic parasternal long-axis view demon Echocardiographic parasternal long-axis view demonstrating a rounded mass in the area of the right ventricular outflow tract. Mass was not causing any outflow obstruction. RV=Right ventricle, LV= Left ventricle, AO=Aorta, Arrow=Right ventricular mass.
  • Left-sided tumors (see image below): With the ability to embolize, these tumors can lead to seizures, transient ischemic attacks, and cerebrovascular and peripheral-vascular accidents.

    Echocardiographic subcostal view of a patient with Echocardiographic subcostal view of a patient with a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. Mass within the left ventricle fills most of the ventricular cavity. LA=Left atrium, RA=Right atrium, RV=Right ventricle, Arrow=Left ventricular mass.
  • Based on their size and position, left-sided and right-sided tumors may interfere with ventricular compliance.

  • Nonproductive cough and hemoptysis have been reported in older children and adults.

  • Malar flush, emboli, spotty hyperpigmentation of the skin, and a distinctive apical diastolic sound called a tumor plop are associated with myxoma.

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Causes

Mutations in the gene protein kinase A (PKA) were identified in patients with Carney complex and myxomas. [6] A mutation of the gene that causes neurofibromatosis is present in patients who have neurofibromatosis and cardiac tumors. Whether or not this means cardiac tumors are directly related to neurofibromatosis is uncertain, although a relationship is likely.

Studies have described the relationship between angiogenesis and tumor growth. [7] Cardiac myxomas produce vascular endothelial growth factor, probably inducing angiogenesis for tumor growth. Neoangiogenesis is involved in the development of masses in the heart, benign or malignant. This knowledge is important for the possible creation of adjuvant therapies for inhibition of the tumor.

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