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 ).
Epidemiology
Its estimated prevalence ranges between <0.01 to 0.33% . There may be slightly increased male predilection .
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 while tricuspid and pulmonary involvement are much rarer.
Very rarely they can also involve the mitral chorda .
Radiographic features
Cardiac MRI
Typically seen as a small (~1.5 cm ) well-defined pedunculated mass in relation to a valve.
- T1: intermediate intensity
- T2: intermediate to high intensity
- 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.
Treatment and prognosis
Surgical resection of the tumor is the treatment of choice.
Complications
- systemic embolization of tumor or thrombotic components
Differential diagnosis
- Lambl’s excrescence
- cardiac myxoma
- valvular thrombi
- valvular vegetations (e.g. due to infective endocarditis or nonbacterial thrombotic endocarditis)
Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu papilläres Fibroelastom des Herzens:
