Cardiac Fibroma

Excerpt

Primary cardiac tumors are rare, having a reported incidence of 0.03% to 0.32%; 75% of these tumors are benign. Among children, cardiac fibromas rank as the second most common primary cardiac tumor after rhabdomyoma. Cardiac rhabdomyomas and fibromas are classified within the subset of nonneoplastic hamartomas. Composed primarily of connective tissue and fibroblasts, cardiac fibromas, although benign, can manifest symptoms due to intracavitary obstruction, inflow and outflow tract obstruction, coronary artery compromise, thromboembolic events, conduction defects, and can even lead to sudden death. Pediatric cardiac tumors often coincide with genetic conditions. For instance, rhabdomyoma is associated with tuberous sclerosis, while approximately 3% to 5% of patients with nevoid basal cell carcinoma syndrome, also known as Gorlin or Gorlin-Goltz syndrome, present with cardiac fibromas.