title: Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience.
abstract: OBJECTIVE
  The aim of this study was to report the results of an international multicenter experience of cardiac magnetic resonance imaging (MRI) evaluation of cardiac tumors in children, each with histology correlation or a diagnosis of tuberous sclerosis, and to determine which characteristics are predictive of tumor type.
  
  
  BACKGROUND
  Individual centers have relatively little experience with diagnostic imaging of cardiac tumors in children, because of their low prevalence. The accuracy of cardiac MRI diagnosis on the basis of a pre-defined set of criteria has not been tested.
  
  
  METHODS
  An international group of pediatric cardiac imaging centers was solicited for case contribution. Inclusion criteria comprised: 1) age at diagnosis ≤18 years; 2) cardiac MRI evaluation of cardiac tumor; and 3) histologic diagnosis or diagnosis of tuberous sclerosis. Data from the cardiac MRI images were analyzed for mass characteristics. On the basis of pre-defined cardiac MRI criteria derived from published data, 3 blinded investigators determined tumor type, and their consensus diagnoses were compared with histologic diagnoses.
  
  
  RESULTS
  Cases (n = 78) submitted from 15 centers in 4 countries had the following diagnoses: fibroma (n = 30), rhabdomyoma (n = 14), malignant tumor (n = 12), hemangioma (n = 9), thrombus (n = 4), myxoma (n = 3), teratoma (n = 2), and paraganglioma, pericardial cyst, Purkinje cell tumor, and papillary fibroelastoma (n = 1, each). Reviewers who were blinded to the histologic diagnoses correctly diagnosed 97% of the cases but included a differential diagnosis in 42%. Better image quality grade and more complete examination were associated with higher diagnostic accuracy.
  
  
  CONCLUSIONS
  Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. rebecca.beroukhim@cardio.chboston.org
authors.forename: Rebecca S
authors.initials: RS
authors.lastname: Beroukhim
authors.forename: Ashwin
authors.initials: A
authors.lastname: Prakash
authors.forename: Emanuela R Valsangiacomo
authors.initials: ER
authors.lastname: Buechel
authors.forename: Joseph R
authors.initials: JR
authors.lastname: Cava
authors.forename: Adam L
authors.initials: AL
authors.lastname: Dorfman
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Festa
authors.forename: Anthony M
authors.initials: AM
authors.lastname: Hlavacek
authors.forename: Tiffanie R
authors.initials: TR
authors.lastname: Johnson
authors.forename: Marc S
authors.initials: MS
authors.lastname: Keller
authors.forename: Rajesh
authors.initials: R
authors.lastname: Krishnamurthy
authors.forename: Nilanjana
authors.initials: N
authors.lastname: Misra
authors.forename: Stephane
authors.initials: S
authors.lastname: Moniotte
authors.forename: W James
authors.initials: WJ
authors.lastname: Parks
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Powell
authors.forename: Brian D
authors.initials: BD
authors.lastname: Soriano
authors.forename: Monvadi B
authors.initials: MB
authors.lastname: Srichai
authors.forename: Shi-Joon
authors.initials: SJ
authors.lastname: Yoo
authors.forename: Jing
authors.initials: J
authors.lastname: Zhou
authors.forename: Tal
authors.initials: T
authors.lastname: Geva
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003951:Diagnostic Errors; D005260:Female; D005350:Fibroma / Q000473:pathology; D006338:Heart Neoplasms / Q000473:pathology*; D006391:Hemangioma / Q000473:pathology; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009206:Myocardium / Q000473:pathology*; D012189:Retrospective Studies; D012207:Rhabdomyoma / Q000473:pathology; D013724:Teratoma / Q000473:pathology