Revision 9 for 'Cardiac fibroma'

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Cardiac fibroma

A cardiac fibroma (also known as cardiac fibromatosis, fibrous hamartoma, or fibroelastic hamartoma) represents a collection of fibroblasts interspersed among large amounts of collagen (some authors believe that this tumor is a hamartoma rather than a neoplasm).

Epidemiology

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 commonest fetal cardiac tumor 2.

Clinical presentation

Cardiac fibromas are often associated with arrhythmias which can be a presenting feature.

Pathology

Cardiac fibromas almost always arise in the ventricles, most commonly in the ventricular septum and left ventricular free wall.

Macroscopic appearance

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. Unlike many primary cardiac tumors, fibromas usually have no foci of cystic change, hemorrhage, or necrosis. Calcification is a common findings.

Associations

There is an increased prevalence of cardiac fibromas in Gorlin syndrome.

Radiographic features

CT

On CT, cardiac fibromas are homogeneous soft-tissue masses that are usually sharply marginated, but also can be infiltrative. Dystrophic calcification is common.

MRI

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.

Cardiac fibromas often demonstrate little or no contrast material enhancement.

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