title: Contrast-enhancement of the anterior eye segment in patients with retinoblastoma: correlation between clinical, MR imaging, and histopathologic findings.
abstract: OBJECTIVE
  AES contrast-enhancement is recognized in a substantial number of retinoblastoma-affected eyes. We retrospectively investigated the histopathologic basis of AES contrast-enhancement on MR images in retinoblastoma.
  
  
  METHODS
  Pretreatment contrast-enhanced MR images were obtained from 42 children with retinoblastoma. Forty-two enucleated eyes were included in this study, AES enhancement was evaluated by using a 3-point score, and these data were correlated with clinical, MR imaging, and histopathologic findings. Additionally, 14 specimens were immunohistochemically analyzed for CD31, VEGF, and Flt-1 expression. Statistical correlations with AES enhancement were assessed by using a linear-by-linear association test and univariate and multivariate ordinal regressions.
  
  
  RESULTS
  The degree of abnormal AES enhancement was moderate in 15 (36%) eyes and strong in 14 (33%) eyes, whereas 13 (31%) eyes showed normal AES enhancement. In multivariate analysis, the degree of AES enhancement showed statistically significant correlations with iris surface-vessel count (P = .05) and optic nerve invasion (P = .04) in the enucleated eye and with tumor volume (P = .02) as detected on MR imaging. No significant associations between AES enhancement and VEGF expression in the iris were observed. Flt-1 (P = .04) staining in iris stroma and IA as detected with CD31 staining (P = .009) both yielded a statistically significant positive correlation with abnormal AES enhancement.
  
  
  CONCLUSIONS
  The degree of abnormal AES enhancement on MR imaging in retinoblastoma reflects angiogenesis in the iris. AES enhancement is also a hallmark of advanced retinoblastoma because its degree correlates with tumor volume and optic nerve invasion.
authors.affiliation: Departments of Radiology, VU University Medical Center, Amsterdam, the Netherlands. p.degraaf@vumc.nl
authors.forename: P
authors.initials: P
authors.lastname: de Graaf
authors.forename: P
authors.initials: P
authors.lastname: van der Valk
authors.forename: A C
authors.initials: AC
authors.lastname: Moll
authors.forename: S M
authors.initials: SM
authors.lastname: Imhof
authors.forename: A Y N
authors.initials: AY
authors.lastname: Schouten-van Meeteren
authors.forename: D L
authors.initials: DL
authors.lastname: Knol
authors.forename: J A
authors.initials: JA
authors.lastname: Castelijns
mesh_terms: D000867:Anterior Chamber / Q000473:pathology*; D001706:Biopsy; D002675:Child, Preschool; D003287:Contrast Media; D015353:Eye Enucleation; D005134:Eye Neoplasms / Q000098:blood supply / Q000378:metabolism / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D009389:Neovascularization, Pathologic / Q000378:metabolism / Q000473:pathology*; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000378:metabolism; D012175:Retinoblastoma / Q000098:blood supply / Q000378:metabolism / Q000473:pathology*; D012189:Retrospective Studies; D042461:Vascular Endothelial Growth Factor A / Q000378:metabolism; D040281:Vascular Endothelial Growth Factor Receptor-1 / Q000378:metabolism
chemical_list: D003287:Contrast Media; D019408:Platelet Endothelial Cell Adhesion Molecule-1; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; C501162:FLT1 protein, human; D040281:Vascular Endothelial Growth Factor Receptor-1