title: How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors?
abstract: BACKGROUND
  Supratentorial atypical teratoid rhabdoid tumor (ATRT) in many cases has a distinctive appearance on post-gadolinium MRI.
  
  
  OBJECTIVE
  We sought to determine whether this is a unique appearance allowing ATRT to be distinguished accurately from other types of pediatric supratentorial tumors.
  
  
  METHODS
  Retrospective review of all available preoperative MRI of pediatric supratentorial tumors at two tertiary children's hospitals, and systematic literature review of case series and reports describing the MRI imaging appearances of supratentorial ATRT.
  
  
  RESULTS
  We had 61 supratentorial tumors, including 32 gliomas, 6 ATRT, 8 ependymomas, 6 gangliogliomas, 2 pilomyxoid astrocytomas, 3 primitive neuro-ectodermal tumors, 2 choroid plexus papillomas, and 2 meningiomas. ATRT presented in significantly younger patients than astrocytomas (mean age 2.6 years vs. 9.9 years, P < 0.05). The visual pattern of a thick, wavy (irregular) heterogeneously enhancing wall around a cystic center was seen in 5/6 (83%) ATRTs and only 3/55 (5.4%) other tumors (P < 0.0001), for specificity of 95%, sensitivity of 83%, positive predictive value of 63% and a negative predictive value of 95%.
  
  
  CONCLUSIONS
  A supratentorial tumor with a thick, wavy (irregular) heterogeneously enhancing wall surrounding a central cystic region is suggestive of ATRT in the appropriate clinical setting, especially in a child of preschool age.
authors.affiliation: Department of Radiology and Diagnostic Imaging, University of Alberta Hospital, 8440-112 Street NW, Edmonton, Canada, T6G 2B7, kong.auyong@gmail.com
authors.forename: Kong Jung
authors.initials: KJ
authors.lastname: Au Yong
authors.forename: Jacob L
authors.initials: JL
authors.lastname: Jaremko
authors.forename: Lennart
authors.initials: L
authors.lastname: Jans
authors.forename: Ravi
authors.initials: R
authors.lastname: Bhargava
authors.forename: Lee T
authors.initials: LT
authors.lastname: Coleman
authors.forename: Vivek
authors.initials: V
authors.lastname: Mehta
authors.forename: Michael R
authors.initials: MR
authors.lastname: Ditchfield
mesh_terms: D000293:Adolescent; D001315:Australia / Q000453:epidemiology; D002170:Canada / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging / Q000706:statistics & numerical data*; D008297:Male; D015203:Reproducibility of Results; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000453:epidemiology* / Q000473:pathology*; D018570:Risk Assessment; D012307:Risk Factors; D012680:Sensitivity and Specificity; D015173:Supratentorial Neoplasms / Q000453:epidemiology* / Q000473:pathology*