title: Occurrence of giant focal forms of congenital hyperinsulinism with incorrect visualization by (18) F DOPA-PET/CT scanning.
abstract: BACKGROUND
  Congenital hyperinsulinism (CHI) is a rare disease characterized by severe hypoglycaemic episodes due to pathologically increased insulin secretion from the pancreatic beta cells. When untreated, CHI might result in irreversible brain damage and death. Currently, two major subtypes of CHI are known: a focal form, associated with local distribution of affected beta cells and a nonfocal form, affecting every single beta cell. The identification of focal forms is important, as the patients can be cured by limited surgery. (18) F DOPA-PET/CT is an established non-invasive approach to differentiate focal from nonfocal CHI.
  
  
  OBJECTIVE
  The purpose of this study was to identify possible limitations of (18) F DOPA-PET/CT scan in patients with focal forms nonfocal CHI.
  
  
  METHODS
  A retrospective chart review of 32 patients (from 2008 through 2013) who underwent (18) F DOPA-PET/CT and partial pancreatectomy for focal CHI at the reference centres in Berlin, Germany and London, UK.
  
  
  RESULTS
  In most cases (n = 29, 90·7%), (18) F DOPA-PET/CT was sufficient to localize the complete focal lesion. However, in some patients (n = 3, 9·3%), (18) F DOPA-PET/CT wrongly visualized only a small portion of the focal lesion. In this group of patients, a so-called 'giant focus' was detected in histopathological analysis during the surgery.
  
  
  CONCLUSIONS
  Our data show that in most patients with focal CHI (18) F DOPA-PET/CT correctly predicts the size and anatomical localisation of the lesion. However, in those patients with a 'giant focal' lesion (18) F DOPA-PET/CT is unreliable for correct identification of 'giant focus' cases.
authors.affiliation: Institut für experimentelle pädiatrische Endokrinologie, Charité Universitätsmedizin, Berlin, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Kühnen
authors.forename: Ritana
authors.initials: R
authors.lastname: Matthae
authors.forename: Ved
authors.initials: V
authors.lastname: Arya
authors.forename: Kathrin
authors.initials: K
authors.lastname: Hauptmann
authors.forename: Karin
authors.initials: K
authors.lastname: Rothe
authors.forename: Simon
authors.initials: S
authors.lastname: Wächter
authors.forename: Michael
authors.initials: M
authors.lastname: Singer
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Mohnike
authors.forename: Traugott
authors.initials: T
authors.lastname: Eberhard
authors.forename: Klemens
authors.initials: K
authors.lastname: Raile
authors.forename: Lina M
authors.initials: LM
authors.lastname: Lauffer
authors.forename: Roman
authors.initials: R
authors.lastname: Iakoubov
authors.forename: Khalid
authors.initials: K
authors.lastname: Hussain
authors.forename: Oliver
authors.initials: O
authors.lastname: Blankenstein
mesh_terms: D002648:Child; D002675:Child, Preschool; D044903:Congenital Hyperinsulinism / Q000175:diagnosis* / Q000601:surgery; D003951:Diagnostic Errors; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D050417:Insulin-Secreting Cells / Q000000981:diagnostic imaging* / Q000473:pathology; D008297:Male; D064847:Multimodal Imaging; D010180:Pancreatectomy; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
chemical_list: D019275:Radiopharmaceuticals; C043437:fluorodopa F 18; D004295:Dihydroxyphenylalanine