Cardiac fibroma, also known as cardiac fibromatosis, are benign congenital cardiac tumors that usually manifest in children.
Cardiac fibroma is a tumor that primarily affects children (most cases is detected in infants or in utero). They are the second most common benign primary cardiac tumor in children after cardiac rhabdomyoma and second most frequent fetal cardiac tumor 2.
Cardiac fibromas are often associated with arrhythmias which can be a presenting feature.
Cardiac fibromas represent a collection of fibroblasts interspersed among large amounts of collagen (some authors believe that this tumor is a hamartoma rather than a neoplasm).
They are well-defined, solitary, intramyocardial lesions with smooth margins and usually large, with a mean diameter of ~5 cm and may obliterate the ventricular cavity. They almost always arise in the ventricles, most commonly in the ventricular septum and left the ventricular free wall. Unlike many primary cardiac tumors, fibromas usually have no foci of cystic change, hemorrhage, or necrosis. Calcification is a common finding.
There is an increased prevalence of cardiac fibromas in Gorlin syndrome.
On CT, cardiac fibromas are homogeneous soft-tissue masses that are usually sharply marginated, but also can be infiltrative. Dystrophic calcification is common.
Due to their dense, fibrous nature, the tumors are usually homogeneous and hypointense on T2-weighted MR images and isointense relative to muscle on T1-weighted images. They often demonstrate little or no contrast material enhancement.