Primary cardiac tumors in children: a 16-year experience

Primary cardiac tumors are rare in children. The majority are benign and noninvasive, although they may have significant hemodynamic consequences depending on their location. The most frequent type in children is rhabdomyoma, usually multiple and intraventricular.

To assess the presentation and outcome of patients with cardiac tumors followed by the Department of Pediatric Cardiology of Hospital de Santa Cruz.

A retrospective analysis was performed of the clinical records of patients diagnosed with primary cardiac tumors between January 1992 and March 2008.

Eleven children with a diagnosis of primary cardiac tumor were identified, three of them detected antenatally. In the remaining cases, median age at diagnosis was 27.5 months, ranging from two days to 13 years. Seven (64%) were male. In three cases diagnosis was made during fetal echocardiography, and in the others by transthoracic echocardiogram. In six patients magnetic resonance imaging was used to confirm the diagnosis. All were benign (eight rhabdomyomas, one papillary fibroelastoma, one myxoma and one pericardial tumor with histological features of Castleman disease). Six patients were asymptomatic at the time of diagnosis; two cases presented with arrhythmias; one with heart failure; and two were diagnosed following stroke, one with right hemiparesis, and the other with paresthesias of the lip and fingers of the right hand. In four cases there was total or partial tumor regression. Surgery was performed in four patients, due to tumor location and/ or significant left ventricular outflow tract obstruction. One of these patients died following surgery due to multiorgan system failure.

Primary cardiac tumors are uncommon in children. Their treatment depends mainly on location and histological type. The most serious complications are ventricular outflow tract obstruction and thromboembolic events, which are associated with a poor prognosis even when surgery is attempted.