Fig. 16.1
Chest x-ray (left) shows cardiomegaly in an infant with tuberous sclerosis and multiple rhabdomyomas. Echocardiogram (middle) shows large, echogenic masses filling the right and left ventricles in the same infant. A sagittal T1-weighted MR image (right) shows a large mass with low signal intensity, which fills the left ventricle
16.2.1 Radiographic Findings
The chest radiograph is normal if there is no hemodynamic compromise. If the mass is large, it can result in signs of congestive heart failure such as pulmonary edema or cardiomegaly.
16.2.2 ECG Findings
ECG may show extrasystoles, ventricular tachycardia, supraventricular tachycardia, or Wolff–Parkinson–White syndrome.
16.2.3 CT Findings
The mass appears hypodense compared with adjacent myocardium. It may project into the ventricular lumen (or less often, the atrial lumen), where it is identified on contrast-enhanced studies.
16.2.4 MRI Findings
T1 isointense/slightly hyperintense
T2 hyperintense
May enhance less than myocardium
No fat suppression
Cine sequence evaluation to assess hemodynamic effects and differentiate lesion from normally contracting myocardium
Homogenous appearance on all sequences
16.2.5 Echocardiography Findings
Solid, hyperechoic, avascular mass
May present as isolated, diffuse wall thickening if lesions are small and multiple
Focal abnormality of cardiac wall motion
Valvular dysfunction possible if mass is large and obstructive
16.2.6 Imaging Recommendations
Echocardiography is the most commonly used modality to detect a small mass. MRI can be helpful to assess function with large masses. The mass is intramyocardial or intracavitary, within the ventricles, and may show additional imaging features of tuberous sclerosis .
16.3 Differential Diagnosis
Fibroma
Teratoma
Rhabdomyosarcoma
Lipoma
Myxoma
Papillary fibroelastoma
Angiosarcoma
16.4 Pathology
This common, benign primary cardiac tumor is a congenital hamartomatous lesion consisting of abnormal myocytes.
16.4.1 Etiology
Given the common feature of spontaneous regression after delivery, maternal hormone regulation may be associated with the development and subsequent growth of the tumor.
16.4.2 Frequency
Rhabdomyomas account for more than 50 % of primary pediatric cardiac tumors. Multiple rhabdomyomas are nearly always associated with autosomal dominant tuberous sclerosis .
16.4.3 Gross Pathologic and Surgical Features
Macroscopic: Solid, round, homogenous mass brighter than adjacent myocardium, most commonly within the ventricular wall. They are usually seen as multiple masses and can have intracavitary extension.
Microscopic: Spindle cells with centrally placed cytoplasm containing the nucleus and myofibrils radiating to the cell wall are pathognomonic; hamartomatous striated muscle fibers.
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