Primary cardiac tumours in the paediatric population Lucio Careddu 1 , Guido Oppido 1 , Francesco Dimitri Petridis 1 , Roberta Liberi 1 , Luca Ragni 2 , Davide Pacini 3 , Carlo Pace Napoleone 1 , Emanuela Angeli 1 and Gaetano Gargiulo 1, * 1 Pediatric Cardiac Surgery and GUCH Unit, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy 2 Pediatric Cardiology and GUCH Unit, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy 3 Adult Cardiac Surgery, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy * Corresponding author. Pediatric Cardiac Surgery and GUCH Unit, S. Orsola-Malpighi Hospital, University of Bologna, Via Massarenti, 9, 40138 Bologna, Italy. Tel: +39 051 636 3156; fax: +39 051 636 3157; e-mail: gaetano.gargiulo@unibo.it (G. Gargiulo). Received 25 April 2013; revised 11 July 2013; accepted 22 July 2013. Summary Primary cardiac tumours are relatively rare in the paediatric population, and they may occur with diferent signs and symptoms in foetal or post-natal life. The clinical manifestations of cardiac tumours in foetal life may include arrhythmias, congestive heart failure and hydrops. In post-natal life, cardiac tumours may cause cyanosis, respiratory distress, myocardial dysfunction, valvular insuiciency, arrhyth- mias, inlow or outlow tract obstructions and sudden death. Surgical treatment is essential when symptoms are present, while the role of medical therapy can merely be palliative. Results are various and related to the patients’ and tumour characteristics. Primary benign heart tumours mainly have a good prognosis, while malignant neoplasms usually have a poor prognosis; in both cases, however, a strict follow- up is always mandatory in order to detect the recurrence of cardiac neoplasms after surgery. Keywords: Cardiac tumours • Pericardial tumours • Survival • Rhabdomyoma • Fibroma • Myxoma INTRODUCTION Primary cardiac tumours are rare in the paediatric population, with a prevalence ranging from 0.0017 up to 0.28% in diferent autopsy series [1, 2]. Furthermore, the incidence of cardiac tumours during foetal life has been reported to be ~0.14% in autopsy series [1, 2]. The vast majority of primary cardiac tumours in children are benign, whereas ~10% are malignant. The fre- quency and type of cardiac tumours in the paediatric population difer from those of adults. In the adult population, primary benign cardiac tumours represent up to 75% of heart neoplasms, while malignant cardiac tumours represent only 25%. Moreover, myxo- mas are the most common primary tumours in adults, accounting for 40% of benign tumours, while, on the contrary, they are exceedingly rare in foetuses and neonates. Rhabdomyoma is the most common cardiac tumour in foetal life and childhood, repre- senting nearly 70% of primary tumours, followed by teratoma, ibroma and haemangioma. Like myxoma, rhabdomyoma is extremely rare in adults. Secondary malignant cardiac tumours are 10–20 times more frequent than primary malignant tumours. The main diagnostic tool in the initial evaluation of cardiac tumours is echocardiography, providing excellent anatomical and functional information. Magnetic resonance imaging (MRI) is the most sensitive examination, being able to characterize tumour tis- sue and vascularity. Moreover, MRI can better deine the relation- ship between cardiac masses and heart chambers and mediastinum structures. MRI could become the main diagnostic tool in the future, with echocardiography, due to its diagnostic characteristics and power. CT scan may also be chosen as an adjunct to both echocardiography and MRI [3]. Currently, the improvement in non-invasive cardiac imaging techniques allows the detection of cardiac tumours even during foetal life and provides the majority of the information needed; however, histology examination of any cardiac masses is manda- tory for a precise diagnosis and a deinitive prognosis. To this pur- pose, endomyocardial biopsy may help histological investigation in selected cases, particularly in right-sided masses. GENERAL CLINICAL FEATURES Cardiac tumours may present either in foetal or post-natal life (Tables 1–3). The manifestations of cardiac tumours in foetal life may include arrhythmias, congestive heart failure and hydrops. In post-natal life, cardiac tumours may cause cyanosis, murmur, respiratory distress, myocardial dysfunction, valvular insuiciency, arrhythmias and sudden death, due to inlow or outlow tract obstructions [6]. Advances in diagnostic imaging with improved tissue characterization and identiication, together with new insights into tumour growth patterns, inluence the treatment strategies for cardiac tumours. The following clinical features are commonly seen with difer- ent cardiac tumours: ● Obstruction. Ventricular tumours may obstruct either the left or the right outlow tract, leading to chest pain, breathlessness or syncope. Atrial tumours, when quite large, may result in the doi:10.1093/mmcts/mmt013 published online 12 September 2013. © The Author 2013. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.