32 Cardiac Tumors
With Christian J. Kellenberger
General Consideration
Cardiac tumors are rare in the pediatric population.
The majority of cardiac tumors are benign.
Categorized by location:
- • Intracavitary
- • Intramural
- • Epicardial
- • Pericardial
- • Paracardiac
Categorized by histologic features and cellular differentiation:
- • Benign tumors
- – Muscle: rhabdomyomas
- – Fibrous tissue: fibromas
- – Fat: lipomas
- – Vascular tissue: hemangiomas
- – Other: myxomas
- – Nervous tissue: paragangliomas
- – Ectopic: teratomas
- • Malignant tumors
- – Mesenchymal: sarcomas
- – Lymphoid: lymphomas
- – Mesothelial: mesotheliomas
Pathophysiology
Rhabdomyomas
- • Most common pediatric cardiac tumor
- • Commonly associated with tuberous sclerosis complex, an autosomal dominant disorder with widespread tumors, called hamartomas, primarily affecting brain, heart, skin, kidneys
- • Most undergo partial or complete spontaneous regression
- • Usually multiple small discrete nodules; rarely a single lesion or rhabdomyomatosis with diffuse myocardial thickening
- • Cystic degeneration and calcification extremely uncommon
- • Conduction system involvement leads to various dysrhythmias.
- • Hemodynamic obstruction uncommon
Fibromas
- • Second most common pediatric cardiac tumor
- • Can be associated with Gorlin basal cell nevus syndrome
- • Spontaneous regression not often seen
- • Usually a large solitary mass
- • Intramural lesion most common locations interventricular septum and left ventricular free wall
- • Cystic degeneration and calcification may be present
- • Associated pericardial effusion not often present
- • Conduction system involvement leads to various dysrhythmias.
- • Hemodynamic compromise due to obstruction to inflow or outflow tracts
- • Coronary arteries may be compressed or incorporated into mass.
Lipomas
- • Large solitary infiltrating and insinuating mass
- • Epicardial lesion on the surface of an atrium or ventricle
- • Coronary arteries may be displaced, compressed, or incorporated into mass.
Hemangiomas
- • Association Kasabach-Merritt syndrome with multiple systemic hemangiomas
- • According to size of vascular channels, divided into capillary, cavernous, or venous types
- • Intracavitary, intramural, epicardial, or pericardial lesion, which can occur in any chamber
- • Calcifications often present
