Primary Pediatric Heart Tumor

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.

Epidemiology

Primary cardiac tumours are rare in paediatric practice with a prevalence of 1.7/1000 to 28/1000 in autopsy series. In contrast, the incidence of cardiac tumours during foetal life has been reported to be approximately 1.4/1000. The incidence of cardiac metastases associated with all types of malignant tumours is estimated to be approximatively 1% (and is 10-20 times higher than primary malignancies of the heart).

Clinical description

The vast majority of primary cardiac tumours in children are benign, whilst approximately 10% are malignant. In contrast, the majority of secondary tumours are malignant. In adults, however, 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. Rhabdomyoma is the most common cardiac tumour during foetal life and childhood. It accounts for more than 60% of all primary cardiac tumours. The manifestations of a cardiac tumour in foetal life include arrhythmia, congestive heart failure, hydrops, and not infrequently stillbirth. In postnatal life cardiac tumours may lead to cyanosis, murmur, respiratory distress, myocardial dysfunction, valvular insufficiency, arrhythmias, and sudden death.

Diagnostic methods

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.

Management and treatment

Surgical resection of primary cardiac tumours should be considered to relieve symptoms and mechanical obstruction to blood flow. 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.

Prognosis

The outcome of surgical resection in symptomatic, non-myxomatous benign cardiac tumours is favourable. The prognosis for malignant primary cardiac tumours is generally extremely poor.