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

Location

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

Very rarely they can also involve the mitral chorda ¹³.

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)

Cardiac CT

  Small hypodense mass with irregular borders.

Echocardiogram

Small pedunculated mass with multiple papillary projections that provides it with the classical appearance of a sea anemone.¹⁴

Treatment and prognosis

Surgical resection of the tumor is the treatment of choice.

Complications

• systemic embolization of tumor or thrombotic components ⁵

Differential diagnosis 

• Lambl’s excrescence
• cardiac myxoma
• valvular thrombi
• valvular vegetations (e.g. due to infective endocarditis or nonbacterial thrombotic endocarditis)