Chapter 487

Primary cardiac tumors are rare at any age. About 90% are benign, and 10% are malignant, most of them sarcomas. These tumors are found mainly in the heart, but can occur in the pericardium or the great arteries. Secondary cardiac involvement from malignant tumors of adjacent organs (lung, thymus) or from kidney (Wilms tumor) or liver, or even remote organs, are also seen. The main types of tumors and age groups at which they occur are shown in eTable 487.1.

eTable 487.1. Main Types of Cardiac Tumors in Children

Most primary tumors have no specific symptoms. They present with pericardial involvement (pain, effusion, tamponade); features of obstruction to blood flow (congestive heart failure, syncope, murmurs, chest pain); conduction defects; arrhythmias (including sudden death); or peripheral embolization. Right-sided tumors may present with pulmonary embolism. Occasionally, tumors are found incidentally during cardiac imaging done for some other reason, including in fetuses.

Diagnosis of suspected tumors is usually by an imaging technique, usually transthoracic echocardiography. It can be supplemented by transesophageal echocardiography, magnetic resonance imaging, and cardiac catheterization and angiography. It is important to remember that an intracardiac mass might be a thrombus or a cyst, rather than a cellular tumor.

Rhabdomyomas are the most common tumors. They are single or multiple, in the ventricles or ventricular septum, and distinguishable from the surrounding compressed myocardium. They usually cause death in children under 5 years of age by obstructing blood flow, but often they regress. They may also cause arrhythmias or heart block. About half of them are associated with the syndrome of tuberous sclerosis, and about half the patients with tuberous sclerosis have cardiac rhabdomyomas. Surgery can be done for isolated tumors.

Fibromas are usually solitary and in the ventricular walls; they are hamartomas. They may cause obstruction but more often cause heart block or ventricular arrhythmias. Surgical excision has been successful.

Papillary fibroelastomas occur predominantly on valves, especially on the left side, and have the typical appearance of papillomas, with long fronds coming from a central stalk, resembling a sea anemone. They frequently embolize.

Myxomas are benign, usually pedunculated tumors that usually arise from the atrial septum near the foramen ovale; occasionally, ...

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