Papillary fibroelastoma diagnosed through multimodality cardiac imaging: a rare tumour in an uncommon location with review of literature

Abstract

We describe the case of a woman presenting with transient ischaemic attack, who was found to have a papillary fibroelastoma arising from the aortic wall, an extremely rare location. We describe the multimodality imaging techniques used in diagnosing this patient and review the most recent literature on evaluation and management of patients with cardiac papillary fibroelastomas.

Surgical removal of an exceedingly rare papillary fibroelastoma of the aortic wall causing unstable angina

“…Given the benign nature of the tumor a simple but accurate excision is sufficient. After surgical resection no recurrence of the tumor has ever been reported 6 .…”

“…The 80% of PFE originate from the valvular surfaces and the remaining 20% from other cardiac localizations; aortic localization is extremely rare and can occur in all aortic segments. To the best of our knowledge, only three cases of aortic wall PFE have been described in literature 3,6,7 .…”

“…In aortic wall PFE, although the majority of patients remain asymptomatic, clinical onset usually occurs with cerebral ischemic or coronary symptoms due to mass embolization or partial or total coronary occlusion 3,4,6,7 . The diagnosis is very difficult mainly due to their low frequency and consequently the lack of knowledge of the case; this is the unique case of aortic PFE reported in our center after about 20,000 cardiac interventions.…”

Surgical removal of an exceedingly rare papillary fibroelastoma of the aortic wall causing unstable angina

“…The 88% of PFE originate from the valvular surfaces and the remaining 12% from other cardiac localizations 6 ; aortic localization is extremely rare and can occur in all aortic segments. To the best of our knowledge, only few cases of aortic wall PFE have been described in literature 3,8,9 …”

“…3,8,9 In aortic wall PFE, although the majority of patients remain asymptomatic, clinical onset usually occurs with cerebral ischemic or coronary symptoms due to embolization or partial or total coronary occlusion. 3,4,8,9 The diagnosis is very difficult mainly due to their low frequency and consequently the lack of knowledge of the case; this is the unique case of aortic PFE reported in our center after about 20,000 cardiac interventions. In addition, presentation with angina symptoms in a patient with previous PTCA caused diagnostic confusion.…”