Discussion
PFE are second in frequency, after myxomas, among primary cardiac tumors, representing about 10%. They present macroscopically with the appearance of a sea anemone with leafy arms, starting from a stalked central core5, consisting of avascular papillary tissue. 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 literature3,6,7.
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 occlusion3,4,6,7. 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. In addiction presentation with angina symptoms in a patient with previous PTCA caused diagnostic confusion. Usually transthoracic echocardiography (TTE) is sufficient to allow a diagnosis with high sensitivity and specificity (88.8% and 88.7% respectively)8 where TEE has even greater sensitivity in smaller PFEs. The execution of a CT scan can be useful to define the diagnosis itself which, due to its extreme rarity, is hardly immediately evaluated. In fact, in our case the typical angina symptoms would probably have led us to perform a new coronary angiogram, with the associated embolic risk, given the difficulty to think of a PFE of the aorta. The presence of the mass, already evident in TTE, led us to the execution of TEE which allowed a diagnosis with high probability; the CT confirmed the size and position of the mass allowing us to place the clinical suspicion and the consequent surgical indication to the removal. Coronary angiography was not performed to avoid mobilization of the mass.
There are no current guidelines for the treatment of PFE. In patients with embolic events, surgery is strongly recommended9. In asymptomatic patients the opinions are different and are based on mass mobility, position, concomitant surgery etc. We believe that, since the tumor is positioned in the aortic flow, surgery should always be indicated for the potential embolic risk, except in asymptomatic highrisk patients. 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 reported6.