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 ||Download (.pdf)
eTable 487.1. Main Types
of Cardiac Tumors in Children
|Type of Tumor||Origin||Main Age Group|
|Rhabdomyoma||Hamartoma||Infant and child|
|Myxoma||Pluripotent mesenchymal cell||Mainly adult|
|Papillary fibroelastoma||Pluripotent mesenchymal cell||Mainly adult|
|Sarcomas||Mesoderm (connective tissue, muscle, fat)||Adult|
|Metastatic tumors||Adult except for Wilms tumor|
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
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, they attach ...