Cardiac and Vascular Tumors in Children

Cardiac tumors are rarely encountered in pediatric practice with a prevalence of 0.0017–0.28 in autopsy series. Benign masses have been reported to make up 90% of primary cardiac tumors in children, and a mere 10% of primary cardiac neoplasms are malignant (McAllister HA Jr. Pathol Annu 14 Pt 2:325–355, 1979; Holley et al. J Am Coll Cardiol 26:516–520, 1995; Nadas and Ellison. Am J Cardiol 21:363–366, 1968; Lam et al. Arch Pathol Lab Med 117:1027–1031, 1993). In contrast, secondary malignant cardiac tumors are 10- to 20-fold more frequent than primary malignant tumors. Rhabdomyoma accounts for more than 60–80% of all primary cardiac tumors in fetal and neonatal life with decreasing incidence in late childhood. Echocardiography, computing tomography, and magnetic resonance imaging all offer unique diagnostic clues to help differentiate type of cardiac tumors. Cardiac catheterization is seldom utilized for diagnostic purpose unless a biopsy is required to define pathological confirmation of a mass. Treatment of cardiac tumors may vary depending on clinical symptoms relating to their natural progression or hemodynamic consequences owing to tumors’ mass effect. Surgical removal of cardiac tumors should be attempted to relieve mechanical obstruction to improve blood flow and consequent symptoms. The effect of surgery in symptomatic patients with nonmyxomatous benign cardiac tumors is favorable. Palliative surgery may be preferred in patients with primary cardiac malignancies as the prognosis for such tumors is universally extremely poor. Similarly, surgical intervention alone is not advocated for patients with metastatic neoplasms. Nevertheless, when it is combined with chemotherapy and radiotherapy surgery may be employed to prolong survival in some cases.