title: Life-threatening tumors of the heart in fetal and postnatal age.
abstract: OBJECTIVE
  To evaluate the role of histology in diagnosis and management of biologically benign heart tumors causing life-threatening symptoms and even death in children and fetuses. The clinical impact of a multidisciplinary approach including 2-D echocardiography, histology, genetics, and cardiac surgery has not yet been fully elucidated.
  
  
  METHODS
  Forty-one consecutive antenatal (n = 17) or postnatal (n = 24) detected cardiac masses were evaluated by 2-D echocardiography (in alive patients) or at autopsy, and 12/41 cases with definite histologic diagnosis of primary and benign cardiac tumor were entered in this study.
  
  
  RESULTS
  Rhabdomyomas (n = 6), hemangiomas (n = 3), central fibrous body chondroma (n = 1), fibroma (n = 1), or left atrial myxoma (n = 1) were histologically diagnosed in 4 fetuses and in 8 children. Death occurred in 6 patients showing diffuse or infiltrative tumors, 2/6 experiencing intrauterine death or sudden and unexpected infant death. Seven patients underwent surgery, 4/7 are alive and well at >5 years follow-up, whereas 3 deaths followed partial tumor resection. Two fetuses with extensive tumor/s were aborted. Tuberous sclerosis complex gene mutations were seen in patients with rhabdomyomas.
  
  
  CONCLUSIONS
  Histology represents the best diagnostic approach in life-threatening pediatric cardiac tumors allowing definite diagnosis in cases other than rhabdomyoma and in sudden deaths, influencing clinical management and counselling. 2-D echocardiography remains the main tool for early clinical diagnosis and follow-up. A multidisciplinary approach is advisable because of rarity, difficult management, and possible associations with inheritable diseases.
authors.affiliation: Department of Anatomic Pathology, University Hospital, Pisa, Italy. angelapucci@libero.it
authors.forename: Angela
authors.initials: A
authors.lastname: Pucci
authors.forename: Gianni
authors.initials: G
authors.lastname: Botta
authors.forename: Noemi
authors.initials: N
authors.lastname: Sina
authors.forename: Maria
authors.initials: M
authors.lastname: Tibaldi
authors.forename: Andrea
authors.initials: A
authors.lastname: Valori
authors.forename: Enrico
authors.initials: E
authors.lastname: Grosso
authors.forename: Andrea
authors.initials: A
authors.lastname: Zonta
authors.forename: Mauro
authors.initials: M
authors.lastname: Giudici
authors.forename: Gabriella
authors.initials: G
authors.lastname: Agnoletti
authors.forename: Laura
authors.initials: L
authors.lastname: Bergamasco
authors.forename: Pietro A
authors.initials: PA
authors.lastname: Abbruzzese
authors.forename: Giovanni
authors.initials: G
authors.lastname: Bartoloni
mesh_terms: D004452:Echocardiography; D005260:Female; D005333:Fetus / Q000473:pathology*; D006338:Heart Neoplasms / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D011379:Prognosis; D016216:Ultrasonography, Prenatal