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DOI: 10.1055/s-0036-1571764
Cardiac Papillary Fibroelastoma Is a Rare Cardiac Tumor with Large Variety of Symptoms: A Retrospective Single-Center Study
Objectives: Papillary fibroelastoma (PFE) is a rare benign primary cardiac tumor and the most frequently found tumor occurring within the stroma of cardiac valves. Overall, this lesion is the second most prevalent primary cardiac tumor beside myxoma. We describe our single-center experience with this entity.
Methods: During the 10-year period between 2005 and 2015, nine patients were treated in our department because of tumorous cardiac masses, which revealed to be a papillary fibroelastoma in histopathological analysis. Seven out of these patients were male, two female. Age ranged between 27 and 75 (mean age 55 years) at the time of surgical procedure.
Results: Most of the patients were diagnosed to have a PFE because of embolic events. Two out of 9 patients were asymptomatic and the tumor was an incidental finding in echocardiography prior to chemotherapy and routine monitoring, respectively. The symptoms of the other 7 patients varied from dyspnea (n = 3) to embolic events (n = 4), most of those cerebral infarction (75%). In the case of a young man of 27 years, detrimental ventricular fibrillation occurred due to embolization of the left main coronary artery as initial symptom. Another patient underwent cardiac surgery because of hypertrophic obstructive cardiomyopathy and a PFE was found in an additional subvalvular membrane as an unusual localization of a PFE. In two cases suspected echocardiographic diagnosis was myxoma of the left atrium, in another case endocarditis of the mitral valve was suspected.
All lesions were resected in total, in some cases followed by reconstruction of cardiac structures depending on the localization of the PFE. There were no death in-hospital and short follow-up (30 days). Moreover, there was no perioperative cerebral infarction, and in only one case the patient developed temporary acute kidney failure. Follow-up by echocardiography showed no evidence of tumor recurrence, yet. Tumor size (median 20 mm) was independent to symptoms and patient age.
Conclusion: Surgical resection and reconstruction if necessary is recommended in symptomatic and asymptomatic patients with cardiac papillary fibroelastoma because excision of the PFE can be safely achieved without evidence of tumor recurrence. Surgical decision making for operation is based on the danger of recurrent cardiac or cerebral embolization and the uncertainty of differential diagnosis of endocarditis or myxoma.