Giant Papillary Fibroelastoma: A Case Report and Review of the Literature

Abstract

Papillary fibroelastomas are rare, benign cardiac tumours that originate from cardiac valves or adjacent endocardium. They have a characteristic appearance that resembles sea anemones with frond-like projections. They are usually asymptomatic until they cause an obstruction to flow (especially coronary ostial flow), or they embolise to cause a stroke. Here we present the case of a 55-year-old female who presented with atypical chest pain and was later discovered to have a large, mobile mass in the ascending aorta side of the noncoronary cusp of the aortic valve, measuring 17 × 15 × 14 mm. This was associated with severe aortic regurgitation. She went on to have an excision of the mass and of her native aortic valve and had a mechanical aortic valve replacement. There were no intraoperative complications and along with an overall unremarkable admission was discharged on postoperative day 5. Histopathology confirmed the diagnosis of papillary fibroelastoma. A systematic review of the literature was conducted in PubMed, searching for the largest papillary fibroelastoma on the aortic valve, with the search term ‘aortic valve papillary fibroelastoma’. The largest measured in the literature was 20 × 15 mm, but this was the combined size of three smaller tumours. This case presents the largest solitary aortic valve papillary fibroelastoma with imaging evidence to our knowledge. Tumours of this size present a unique challenge to the cardiac surgeon in their surgical management.