Statistics from Altmetric.com
Papillary fibroelastoma of the mitral valve does not occur frequently, with fewer than 50 cases being reported in the literature. Therefore each case is interesting to report in order to improve the diagnosis and management of this uncommon tumour. We report an image of a fibroelastoma of the mitral valve chordae, without mitral insufficiency, discovered accidentally.
Papillary fibroelastoma was first described as gigantic Lambl’s excrescences. In typical cases papillary fibroelastoma resembles a sea anemone with multiple papillary fronds attached to the endocardium by a short pedicle. Primary tumours of the heart and pericardium are extremely rare, with an incidence between 0.0017−0.28% in reported or collected necropsy series. More than 70% of these tumours are benign, and only 8% are papillary fibroelastomas, mostly located on heart valves. Cardiac primary fibroelastoma (CPFE) is by far the most common tumour arising from cardiac valves (about 70–80%). CPFEs are small, avascular, benign tumours. The most frequent location is the valvar endocardium of the aortic valve (30%) and the mitral valve (20–25%) Only 10% are non-valvar. Because of their potential for cerebral and coronary embolisation, even small papillary fibroelastomas should be excised, even in asymptomatic patients.