Cardiac fibroma, fibroma

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07/14/23

Cardiac fibroma is a benign neoplasm primarily of infants and children. It is the second most common in this age group. Rhabdomyoma is the most common benign cardiac tumor of childhood. Fibromas are still very rare with only about 100 reported cases since 1976.
There is an increased incidence of cardiac fibromas in Gorlin syndrome (nevoid basal cell carcinoma syndrome). This syndrome demonstrates autosomal dominant inheritance and is associated with basal cell carcinoma, odontogenic keratocysts of the mandible, skeletal anomalies, and neoplastic growth in several organ systems.
One-third of patients are asymptomatic and are detected incidentally, but patients may present with heart failure, chest pain, arrhythmia, and sudden cardiac arrest. Because of the risk of sudden death, surgical removal is recommended, even in asymptomatic patients.

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