Cardiac tumours in children.Orphanet J Rare Dis. 2007 Mar 1;2:11.

Cardiac tumours are benign or malignant neoplasms arising primarily in the inner lining, muscle layer, or the surrounding pericardium of the heart. They can be primary or metastatic. Primary cardiac tumours are rare in paediatric practice with a prevalence of 0.0017 to 0.28 in autopsy series. In contrast, the incidence of cardiac tumours during foetal life has been reported to be approximately 0.14%. The vast majority of primary cardiac tumours in children are benign, whilst approximately 10% are malignant. Secondary malignant tumours are 10-20 times more prevalent than primary malignant tumours. Rhabdomyoma is the most common cardiac tumour during foetal life and childhood. It accounts for more than 60% of all primary cardiac tumours. The frequency and type of cardiac tumours in adults differ from those in children with 75% being benign and 25% being malignant. Myxomas are the most common primary tumours in adults constituting 40% of benign tumours. Sarcomas make up 75% of malignant cardiac masses. Echocardiography, Computing Tomography (CT) and Magnetic Resonance Imaging (MRI) of the heart are the main non-invasive diagnostic tools. Cardiac catheterisation is seldom necessary. Tumour biopsy with histological assessment remains the gold standard for confirmation of the diagnosis. Surgical resection of primary cardiac tumours should be considered to relieve symptoms and mechanical obstruction to blood flow. The outcome of surgical resection in symptomatic, non-myxomatous benign cardiac tumours is favourable. Patients with primary cardiac malignancies may benefit from palliative surgery but this approach should not be recommended for patients with metastatic cardiac tumours. Surgery, chemotherapy and radiotherapy may prolong survival. The prognosis for malignant primary cardiac tumours is generally extremely poor.

Cardiac rhabdomyoma with long-term conduction abnormality: progression from pre-excitation to bundle branch block and finally complete heart block.Med Sci Monit. 2007 Feb;13(2):CS21-3.

BACKGROUND: Cardiac rhabdomyoma, a benign smooth muscle hamartoma, is the commonest heart tumor encountered in children. Arrhythmias and conduction disorders have been described with these tumors, although most electrocardiographic abnormalities will resolve over time, concomitant with the resolution of the tumor. CASE REPORT: The case of a child, diagnosed with cardiac rhabdomyomas in fetal life, is reported in whom the tumors regressed in size over a four-year period whilst displaying a spectrum of conduction disturbances. He finally developed potentially lethal complete heart block. CONCLUSIONS: Cardiac rhabdomyomas may be asymptomatic, cause hemodynamic compromise, or be associated with rhythm disorders. In the presence of conduction disorders, close monitoring is required with regular electrocardiograms to detect potential lethal conduction disorders.