title: Cardiac tumors: the 17-year experience of pediatric cardiology department.
abstract: BACKGROUND
  Primary cardiac tumork may be benign or malignant and may arise anywhere in the endocardium, myocardium or pericardium. They are rare in pediatric age-groups, with a prevalence in autopsy findings of 0.0017 to 0.28%; most are benign, malignant tumors accounting for only 10% of the total.
  
  
  OBJECTIVE
  The purpose of this paper is to report the 17-year (1989-2006) experience of primary cardiac tumors in a single center. The clinical data and histopathological diagnoses were collected from a retrospective chart review.
  
  
  RESULTS
  We identified 12 patients, with a mean age at diagnosis of 23 months, in two cases diagnosed in the fetal period. The frequent cause of referral was tuberous sclerosis, followed by a cardiac murmur. The diagnosis of primary cardiac tumor was based on echocardiography and/or magnetic resonance imaging. The patients all underwent 24-hour electrocardiographic Holter monitoring, and the most common finding was nonspecific repolarization abnormalities. The most frequent cardiac tumor was rhabdomyoma (67%), usually associated with tuberous sclerosis, followed by fibroma (17%) and fibroelastoma (8%) Although biopsy is the diagnostic gold standard, it was only performed in two patients. Surgical removal of the tumor was performed in a single patient (with fibroelastoma) due to the perceived risk of pulmonary embolization.
  
  
  CONCLUSIONS
  In our population, the most frequent tumor was rhabdomyoma associated with tuberous sclerosis. Most patients were asymptomatic, and the diagnosis was made in routine observations on patients with tuberous sclerosis or with a cardiac murmur. A histopathological diagnosis is only warranted in the rarest forms of cardiac tumors.
authors.affiliation: Serviço de Cardiologia Pediátrica, Centro Hospitalar de Lisboa Central-Hospital de Santa Marta, Lisboa, Portugal. f_parames@hotmail.com
authors.forename: Filipa
authors.initials: F
authors.lastname: Paramés
authors.forename: Isabel
authors.initials: I
authors.lastname: Freitas
authors.forename: José Diogo Ferreira
authors.initials: JD
authors.lastname: Martins
authors.forename: Conceição
authors.initials: C
authors.lastname: Trigo
authors.forename: Maria Fátima F
authors.initials: MF
authors.lastname: Pinto
mesh_terms: D002309:Cardiology; D002648:Child; D005260:Female; D006338:Heart Neoplasms / Q000175:diagnosis / Q000628:therapy; D006748:Hospital Departments; D006801:Humans; D007223:Infant; D008297:Male; D010372:Pediatrics; D012189:Retrospective Studies