Multifocal "tapete" papillary fibroelastoma

Abstract

Papillary fibroelastomas (PFEs) are benign cardiac tumours that typically arise on diseased aortic or mitral valves. Usually solitary, PFEs are characterised by numerous frond-like branches attached to the valve surface by a single stalk, elastic tissue present within the central core, and the appearance of a sea anemone when immersed in water. PFEs have a predilection for men with a mean age of 56 years. A very rare case of a 25-year-old man with multiple lesions on the aortic and mitral valves and a large confluent "carpet-like" lesion in the left ventricular cavity is presented. Admitted with a significant left middle cerebral artery stroke and hemiplegia, investigations showed a left ventricular outflow tract lesion, with surgical excision displaying a multifocal "carpet-like" PFE. Histopathological examination showed various lesions presenting with a short stem, several fronds arising from each stem like a "tree trunk", and elastic tissue within the central core. This case is presented, along with a review of 833 reported cases of PFEs published after the year 2001. It is believed that this is the first reported instance of surgically resected and morphologically confirmed multiple PFEs seen and removed at one operation.