PAPILLARY FIBROELASTOMA: AN UNUSUAL CAUSE OF TRANSIENT ISCHEMIC ATTACK

Abstract

Abstract Papillary fibroelastomas (PFEs) are small, avascular benign tumors. Despite that, PFEs could lead to important clinical consequences such as transient ischemic attack and myocardial infarction. A 65–year–old female patient was admitted to our ER presenting a transient ischemic attack characterized by retrograde amnesia lasting about an hour. She had a history of systemic scleroderma and left breast cancer treated with local radiotherapy. In our hospital she underwent a neurological examination and a head CT which were negative for ongoing acute pathologies. An electrocardiogram was performed which showed sinus rhythm at 65 bpm. A transthoracic echocardiogram was also performed revealing a floating mass arising from left ventricular outflow tract between aortic and mitral valves. The transesophageal echocardiogram confirmed a solitary lesion approximately 2 centimeters long localized between A2 and A3 mitral valve scallops. The patient was therefore referred for surgery. During surgery the mass was found to arise from anterior mitral annulus and was resected. The histopathological examination of the mass confirmed the morphologic diagnosis of papillary fibroelastoma. Histologically PFEs are made up of a single layer of endocardial cells covering a matrix of collagen. The main risk factors for the development of PFEs are iatrogenic causes, such as radiotherapy, and autoimmune diseases such as systemic scleroderma. In 54% of cases PFEs are asymptomatic at the time of diagnosis. Since over 95% of PFEs present on the left side of the heart, systemic embolism is the most frequent presentation typically as transient ischemic attacks. Initial evaluation is accomplished with echocardiography. PFEs appear as echo–dense and pedunculated masses. Cardiac computed tomography and magnetic resonance imaging are second line modalities in PFEs evaluation. Definitive diagnosis of PFE requires pathological confirmation. Surgical resection remains the treatment of choice for all symptomatic patients. For symptomatic patients who are not surgical candidate, long term antiplatelets or anticoagulation therapy should be initiated. Asymptomatic patients should have surgical resection if the size of the tumor is > 1 cm. PFEs are associated with significant morbidity and mortality. Echocardiography remains the most utilized diagnostic modality. Surgical treatment should always be considered. Antithrombotic therapy should be considered in non–surgical candidates.