Background:
Cardiac tumors are classified by histology and site of origination. These fall into three broad categories: primary cardiac tumors, solid tumors that have spread to the heart, and infradiaphragmatic tumors that extend into the heart.1
Primary cardiac tumors (PCTs) are rare, with an incidence between 0.001-0.28%.1-4 Seventy five percent (75%) are benign, with myxoma representing the most common histology. Papillary fibroelastoma (PFE) is the third most common benign cardiac tumor; but recent evidence suggests that its incidence may be higher.5-9
This article reviews PFE, describing tumor histology and biology, clinical manifestations, diagnostic and imaging work-up, and surgical treatment options for both valvular- and ventricular-based tumors.