- Papillary fibroelastoma (PFE) is a rare and histologically benign tumor, but it may have malignant propensity for life-threatening complications. Herein are described four cases of PFE which reflect the clinical diversity of this lesion. The diagnostic and surgical approach utilized is also briefly reviewed. The files of four patients with cardiac valvular PFE treated at the authors' center between January 2002 and November 2003 were reviewed. The diagnosis was strongly suggested by echocardiography. Tumors were noted in aortic (n = 2), mitral (n = 1) and tricuspid (n = 1) sites. Indications for surgery were myocardial infarction (both aortic tumors), previous stroke (mitral tumor), and preventive (tricuspid tumor). Surgical excision with a conservative, valve-sparing approach was performed in all cases. For the first aortic tumor, the aortic valve was reconstructed with a patch of autologous pericardium. None of the patients had evidence of valvular regurgitation after excision on intraoperative transesophageal echocardiography, and all had an uneventful recovery. There were no cases of recurrence or regurgitation on follow up echocardiography. PFE is an uncommon but increasingly recognized cause of embolic phenomena. Prompt identification allows for surgical excision, which seems to be curative, safe and well-tolerated. A conservative valve-sparing approach is recommended because of the absence of recurrence after total excision.