Revision 13 for 'Cardiac fibroma'

All Revisions

Cardiac fibroma

Cardiac fibroma, also known as cardiac fibromatosis, are benign congenital cardiac tumors that usually manifest in children. 

Epidemiology

Cardiac fibromas are tumors that primarily affect children (most cases are detected in infants or in utero) with a ratio of 4:1 compared with adults 5. They are the second most common benign primary cardiac tumor in children after cardiac rhabdomyoma and second most frequent fetal cardiac tumor 2.

Clinical presentation

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

Pathology

Microscopic appearance

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).

Macroscopic appearance

These tumors 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 left ventricular free wall, interventricular septum or right ventricular free wall (in descending order) 5.

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

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 seen in approximately 25% of cases 5. These lesions may enhance homogeneously or heterogeneously after administration of contrast. 

MRI

Due to their dense, fibrous nature, the tumors are usually hypointense on T2-weighted MR images and isointense relative to muscle on T1-weighted images. They are usually homogeneous unless there are calcifications, which may be seen as patchy hypointense foci within the lesion. Because of their avascularity, they often demonstrate little or no contrast enhancement.

Differential diagnosis

  • rhabdomyoma
    • also seen in young patients
  • rhabdomyosarcoma
    • large and heterogeneous mass
    • invasive
  • myxoma
    • intracavitary rather than intramural
  • metastases 
    • known primary neoplasm
    • less likely in young patients
    • multifocal masses 

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.