Papillary Fibroelastoma

Cardiac tumors in children are rare, with an autopsy frequency of 0.001% to 0.30%. Most primary cardiac tumors are benign, with rhadomyoma, myxoma, teratoma, and fibroma being most common. Papillary fibroelastoma is the third most common tumor in adults but is very rare in children, with an incidence of 0.13% in autopsy series. These tumors typically involve left-sided valvular structures, and can be associated with serious complications such as myocardial infarction, stroke, and sudden death. Typical clinical presentations can range from asymptomatic patients to those with sequelae from outflow tract obstruction, or systemic or pulmonary emboli. Definitive management of large cardiac tumors in children involves complete surgical resection. 


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Karimi M, Vining M, Pellenberg R, Jajosky R. Papillary fibroelastoma of tricuspid valve in a pediatric patient. Ann THorac Surg 2013;96:1078-80.
Unusual Cancers of Childhood Treatment (PDQ(R)): Health Professional Version. PDQ Cancer Information Summaries. Bethesda (MD) 2016.