title: Lesions simulating retinoblastoma (pseudoretinoblastoma) in 604 cases: results based on age at presentation.
abstract: OBJECTIVE
  To determine the types and frequency of ocular conditions that simulate retinoblastoma (pseudoretinoblastoma) based on age at presentation.
  
  
  METHODS
  Retrospective case series.
  
  
  METHODS
  Two thousand seven hundred seventy-five patients.
  
  
  METHODS
  Chart review.
  
  
  METHODS
  Conditions simulating retinoblastoma.
  
  
  RESULTS
  Of 2775 patients referred for management of retinoblastoma, 2171 patients (78%) had confirmed retinoblastoma and 604 patients (22%) had simulating lesions (pseudoretinoblastomas). In the pseudoretinoblastoma cohort, the mean patient age at presentation was 4 years (median, 2 years). There were 27 different pseudoretinoblastoma conditions, and the 10 most common included Coats' disease (n = 244; 40%), persistent fetal vasculature (PFV; n = 158; 28%), vitreous hemorrhage (n = 27; 5%), ocular toxocariasis (n = 22; 4%), familial exudative vitreoretinopathy (FEVR; n = 18; 3%), rhegmatogenous retinal detachment (n = 18; 3%), coloboma (n = 17; 3%), astrocytic hamartoma (n = 15; 2%), combined hamartoma of retina and retinal pigment epithelium (n = 15; 2%), and endogenous endophthalmitis (n = 10; 2%). Simulating lesions differed based on age at presentation, and children younger than 1 year were most likely to have PFV (49%), Coats' disease (20%), or vitreous hemorrhage (7%); those 2 to 5 years of age were most likely to have Coats' disease (61%), toxocariasis (8%), or PFV (7%); and those older than 5 years were most likely to have Coats' disease (57%), toxocariasis (8%), or FEVR (6%).
  
  
  CONCLUSIONS
  The most common pseudoretinoblastomas include Coats' disease, PFV, and vitreous hemorrhage, but the spectrum varies depending on patient age.
authors.affiliation: Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA. carol.shields@shieldsoncology.com
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Schoenberg
authors.forename: Kristen
authors.initials: K
authors.lastname: Kocher
authors.forename: Shripaad Y
authors.initials: SY
authors.lastname: Shukla
authors.forename: Swathi
authors.initials: S
authors.lastname: Kaliki
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D054514:Persistent Hyperplastic Primary Vitreous / Q000175:diagnosis*; D012164:Retinal Diseases / Q000175:diagnosis*; D019572:Retinal Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging; D012175:Retinoblastoma / Q000175:diagnosis* / Q000000981:diagnostic imaging; D012189:Retrospective Studies; D014463:Ultrasonography; D014823:Vitreous Hemorrhage / Q000175:diagnosis*; D055815:Young Adult