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.