Cardiac Fibroma

Introduction
• It is benign tumor of fibroblasts
• Second most common primary cardiac tumor in infants and children
• 90% in children <1 year
• Slight male predominance
• Present in solitary mass
• Commonly found in the LV septum or the LV free wall
• Occurrence in the RV or the atria in less than 10% of cases
• Does not regress as rhabdomyoma
• The differential feature favoring fibroma over rhabdomyoma is
calcification, which occurs in fibromas but not in rhabdomyomas
Etiology
• Cardiac fibroma may be a manifestation of Gorlin syndrome, which is
caused by mutations in PTCH1.
• Homozygous and heterozygous losses of the PTCH1 gene is in sporadic
cardiac fibromas.
Pathogenesis
• The exact pathogenesis of disease is not completely understood
• Typically tumor of CHILDREN, most often in first year of life.
• Benign tumor of fibroblasts; may be locally infiltrative.
• Most often in ventricles (left>right) or ventricular septum.
• Septal involvement and age less than 17 years old at the time of
diagnosis are associated with a poor prognosis.
Signs and Symptoms
• Heart failure due to mechanical obstruction is the most common
symptom.
• cyanosis, arrhythmias, and syncope
• Also causes myocardial dysfunction.
• 1/3rd of patients with cardiac fibroma can remain asymptomatic.
Histopathology
• Cardiac fibroma is composed of monomorphic fibroblasts that
demonstrate little or no atypia.
• The margins infiltrate into cardiac muscle.
• The degree of cellularity often decreases with the age of the patient,
and the amount of collagen increases.
• Mitoses are generally present only in tumors occurring in infants.
• Calcification is a common finding with these fibromas in patients of all
ages.
Grossly
• They typically appear as subendocardial yellow-tan nodules or
plaques.
• The average size of the tumor is 5 cm
• Firm, circumscribed, pale tan, intramural, or ventricular cardiac mass
• The cut surface displays a firm texture
• Satellite nodules may accompany the solitary mass
• As the tumor matures, the degree of collagen increases such that in
elderly patients it resembles scar
Cardiac Fibroma. A cardiac septal fibroma displaces myocardium and has a fleshy
yellow and white cut surface.
Microscopic Appearance
• Histologically, bland fibroblasts intermingle with collagen and elastic
fibers
• May infiltrate into surrounding myocardium.
Cardiac Fibroma. Bland spindle cells form large fascicles and lack hypercellularity
or nuclear atypia.
(a) The tumor is a bulging firm white mass that distorting the surrounding myocardium. This tumor obliterated
the left ventricle and ventricular septum; note the tricuspid valve between the right atrium and the ventricle
(arrow). (b) A trichrome stain demonstrates infiltration of the collagenous tumor into cardiac muscle (Masson-
trichrome). (c) In children, cardiac fibromas may be markedly cellular, similar in appearance to fibrosarcoma.
(d) In adults, the tumors are composed almost entirely of nearly acellular collagen (c and d, H&E stain).
Treatment
• Complete surgical resection in symptomatic cases is recommended.
• The postoperative prognosis is typically good.
• For large, unresectable tumors, cardiac transplantation may be
considered.