title: Central nervous system imaging in childhood Langerhans cell histiocytosis - a reference center analysis.
abstract: BACKGROUND
  The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation.
  
  
  METHODS
  We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed.
  
  
  RESULTS
  Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (κ > 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary.
  
  
  CONCLUSIONS
  The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials.
authors.affiliation: Neuroradiology Department, Johann Wolfgang Goethe University, Frankfurt/Main, Germany.
authors.forename: Luciana
authors.initials: L
authors.lastname: Porto
authors.affiliation: Pediatric Hematology and Oncology, Hospital for Children and Adolescents, Johann Wolfgang Goethe University, Frankfurt/Main, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Schöning
authors.affiliation: Neuroradiology Department, Johann Wolfgang Goethe University, Frankfurt/Main, Germany.
authors.forename: Elke
authors.initials: E
authors.lastname: Hattingen
authors.affiliation: Pediatric Hematology and Oncology, Hospital for Children and Adolescents, Johann Wolfgang Goethe University, Frankfurt/Main, Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Sörensen
authors.affiliation: Neuroradiology Department, Johann Wolfgang Goethe University, Frankfurt/Main, Germany.
authors.forename: Alina
authors.initials: A
authors.lastname: Jurcoane
authors.affiliation: Pediatric Hematology and Oncology, Hospital for Children and Adolescents, Johann Wolfgang Goethe University, Frankfurt/Main, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Lehrnbecher
keywords: Langerhans cell histiocytosis; central nervous system; child; magnetic resonance imaging