Revision 12 for 'Papillary fibroelastoma of the heart'

All Revisions - View changeset

Papillary fibroelastoma of the heart

Papillary fibroelastomas are rare benign primary cardiac tumors. However, of the primary cardiac tumors, they are one of the commonest to occur in relation to the cardiac valves (may account for 75% of valvular tumors 10). 

Epidemiology

Its estimated prevalence ranges between <0.01 to 0.33% 5. There may be slightly increased male predilection 10.

Pathology

Characterized by papillary growth of stromal tissue composed of a central dense amorphous fibrous core and an surrounding layer of loose connective tissue. There is a also a layer of elastic fibers (hallmark) which is variable in distribution. This is further surrounded by a single layer of hypertrophied endothelium. 

Location

~80% occur in relation to either the aortic or mitral valve 2 while tricuspid and pulmonary involvement is much rarer.

Radiographic features

Cardiac MRI 

Typically seen as a small (~1.5 cm 5) well defined pedunculated mass in relation to a valve.

  • T1: intermediate intensity 1-2
  • T2: intermediate to high intensity 1-2
  • SSFP Cine: may be useful for showing mobility (often highly mobile)

 Complications

  • systemic embolization of tumor or thrombotic components 5

Treatment 

Surgical resection is the treatment of choice.

Updating… Please wait.

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

 Thank you for updating your details.