Left atrial myxoma with papillary fibroelastoma-like features

Although rare, papillary fibroelastoma (PFE) of the heart valves and atrial myxoma represent the two most common cardiac tumors. Coexistence of these two lesions has been documented in rare case reports. We describe the case of a 77-year-old man who presented with slightly progressive chest pain associated with dyspnea, fatigue and edema of the lungs. Transthoracic echocardiography detected a left atrial mass that has been successfully excised. Histopathological examination showed a neoplasm combining features of both atrial myxoma and PFE. However, close evaluation of the latter showed microscopic foci of myxomatous tissue within papillary cores, indicating that the PFE-like component has developed around preexisting myxomatous tissue that served as a nidus for papillary fronds, probably by a process of fibrinous microthrombosis, organization and endothelialisation. This unusual case may shed light on the pathogenesis of the PFE pattern.