ABSTRACT
A1 C Cardiac tumours are classified as primary (arising from the heart, more than 90% of which are benign) or secondary (representing metastases, which are uniformly malignant). Most cardiac tumours in children and adults represent metastatic disease. In children, these are secondary to non-Hodgkin’s lymphoma, leukaemia and neuroblastoma. The most common primary cardiac tumour in children is rhabdomyoma, accounting for 50%–80% of all paediatric primary cardiac tumours. Other benign primary cardiac tumours vary according to age: in neonates and infants, fibroma and intrapericardial teratoma may occur, while in older children and adolescents, myxoma and fibroma are seen. Rhabdomyomas are multiple, well-circumscribed, white, intracavitary or intramural masses that may occur anywhere in the heart. Most are diagnosed in the newborn period and may present with respiratory distress or heart failure, especially if large. Tuberous sclerosis, an autosomal dominant mutation with variable expressivity, is a common association, with approximately 50% of patients with tuberous sclerosis having rhabdomyomas. Approximately 30% of rhabdomyomas will regress spontaneously. Rhabdomyosarcoma is the most common primary malignant cardiac tumour in children whereas angiosarcoma is the most common primary malignant cardiac tumour in adults; both portend a poor prognosis. Myxomas, arising from the interatrial septum, are the most common primary benign cardiac tumours in adults, usually diagnosed in the third to sixth decade of life. Treatment is by surgical excision with reconstruction of the interatrial septum. Fibromas represent the second most common primary benign cardiac tumour in children,
and present usually as solitary large white non-encapsulated tumours in the left ventricular septum or free wall. Presentations include left ventricular outflow tract obstruction and ventricular tachyarrhythmias. Treatment is by surgical enucleation on cardiopulmonary bypass. Intrapericardial teratomas are solitary, encapsulated tumours that are attached to the base of the heart. As with other teratomas, all three embryonic germ cell layers may be present. Treatment is by surgical excision.
