title: Medical and surgical management of primary cardiac tumours in infants and children.
abstract: Primary cardiac tumours in infants and children are extremely rare, with an estimated incidence of 0.2% according to echocardiographic studies. Owing to their rarity, there is very little literature available, and most knowledge is based on collections of case reports. Therefore, we reviewed retrospectively our 27 years of clinical experience on the overall management of cardiac tumours among children in order to improve not only our knowledge but also to provide others with information about the incidence, clinical presentation, management, and long-term outcome of this rare disease. Between April, 1982 and April, 2009, 52 children were diagnosed with cardiac tumours at our Institution. Medical records and follow-up echocardiographic evaluations were studied. The diagnosis was prenatal in 35% of the patients. The most frequent tumour types were rhabdomyomas (61.5%), fibromas (15.4%), and myxomas (5.8%). There were no cases of primary malignant tumours. All diagnoses were achieved using two-dimensional echocardiography, and for 12 patients a pathological analysis was carried out. A total of 41 patients (79%) were managed medically, whereas 11 (21%) patients underwent surgical treatment. At a mean follow-up of 7.2 ± 5.4 years, two patients died of complications related to cardiac transplantation; all the remaining patients are in excellent clinical condition. In conclusion, cardiac tumours in paediatric practice are usually clinically and histologically benign. Only a few cases need surgery. Up to one-third of the cardiac masses are detectable prenatally. Rhabdomyoma is the most common histotype, followed by fibroma and myxoma. The long-term prognosis is generally good.
authors: 1 Department of Cardiac, Pediatric and Congenital Cardiac Surgery Unit, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy.
authors: Massimo A
authors: MA
authors: Padalino
authors: 2 Department of Pediatrics, Division of Pediatric Cardiology, University of Padova Medical School, Padova, Italy.
authors: Elena
authors: E
authors: Reffo
authors: 2 Department of Pediatrics, Division of Pediatric Cardiology, University of Padova Medical School, Padova, Italy.
authors: Alessia
authors: A
authors: Cerutti
authors: 2 Department of Pediatrics, Division of Pediatric Cardiology, University of Padova Medical School, Padova, Italy.
authors: Valentina
authors: V
authors: Favero
authors: 2 Department of Pediatrics, Division of Pediatric Cardiology, University of Padova Medical School, Padova, Italy.
authors: Roberta
authors: R
authors: Biffanti
authors: 1 Department of Cardiac, Pediatric and Congenital Cardiac Surgery Unit, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy.
authors: Vladimiro
authors: V
authors: Vida
authors: 1 Department of Cardiac, Pediatric and Congenital Cardiac Surgery Unit, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy.
authors: Giovanni
authors: G
authors: Stellin
authors: 2 Department of Pediatrics, Division of Pediatric Cardiology, University of Padova Medical School, Padova, Italy.
authors: Ornella
authors: O
authors: Milanesi
mesh_terms: D000293:Adolescent; D001145:Arrhythmias, Cardiac / Q000188:drug therapy / Q000209:etiology; D058070:Asymptomatic Diseases; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004452:Echocardiography; D005260:Female; D005350:Fibroma / Q000000981:diagnostic imaging / Q000628:therapy*; D006338:Heart Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009232:Myxoma / Q000000981:diagnostic imaging / Q000628:therapy*; D009365:Neoplasm Regression, Spontaneous; D009378:Neoplasms, Multiple Primary / Q000000981:diagnostic imaging / Q000628:therapy*; D011247:Pregnancy; D012189:Retrospective Studies; D012207:Rhabdomyoma / Q000000981:diagnostic imaging / Q000628:therapy*; D013724:Teratoma / Q000000981:diagnostic imaging / Q000628:therapy*; D016216:Ultrasonography, Prenatal