16 June 2020 : Case report
Papillary Fibroelastoma of the Right Ventricle in the Setting of Multifocal Pulmonary Hemorrhages and Pulmonary Embolization in a 39-Year-Old Man: A Case Report
Challenging differential diagnosis, Rare diseaseShahnaz Duymun1EF*, Asad Nasir1E
Am J Case Rep 2020; 21:e923074
BACKGROUND: Papillary fibroelastoma accounts for approximately 24% of all primary cardiac tumors and is the third most common primary cardiac neoplasm. It mostly involves the cardiac valves; however, there have been cases of involvement of the endocardium. Papillary fibroelastoma, although typically benign, can cause a plethora of complications, including systemic embolization, myocardial infarction, arrhythmia, and sudden cardiac death. In this article we present a unique case of a man diagnosed with a papillary fibroelastoma of the right ventricle in the setting of multifocal pulmonary hemorrhages and pulmonary embolization.
CASE REPORT: A 39-year-old man presented with hemoptysis and dyspnea due to bilateral lower-lobe pneumonia and a left pleural effusion. Further imaging throughout his hospitalization revealed worsening multifocal consolidations, pulmonary hemorrhages, and a pulmonary embolus. A mobile cardiac mass measuring 30.2×20 mm, detected by echocardiography, was found in the right ventricle, partially fixed to the intraventricular septum via a 14.4-mm stalk, which was surgically excised and was found to be consistent with papillary fibroelastoma. He underwent an IVC filter and was discharged on warfarin, which he tolerated well.
CONCLUSIONS: Papillary fibroelastomas are benign infrequent findings but carry a high risk of systemic complications, as demonstrated in our patient. He had no known cardiac disease or risk factor for tumor growth, yet developed a papillary fibroelastoma of the right ventricle, which is rare. Given the potential of fatal outcomes, it is imperative that patients with unexplained embolic phenomena undergo early diagnosis by echocardiography and early surgical treatment.
Keywords: Cardiac Surgical Procedures, Heart Neoplasms, Pulmonary Embolism, Cardiac Papillary Fibroelastoma, Dyspnea, Echocardiography, Heart Ventricles, Hemoptysis, Hemorrhage
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