title: Characteristics of Germline and Non-germline Retinoblastomas.
abstract: OBJECTIVE
  To discuss the clinical characteristics, treatment and outcomes of germline and non-germline retinoblastoma tumors.
  
  
  METHODS
  A retrospective study was performed on retinoblastoma cases from 1979 to 2007. General characteristics of the patients, treatment modalities, histopathological findings and survival were compared in germline versus non-germline cases.
  
  
  RESULTS
  We analyzed 557 cases of retinoblastoma with mean age of 32.2±22.0 months including 177 and 380 patients with germline and non-germline tumors, respectively. Germline cases were significantly different from non-germline counterparts in terms of mean age (24.7±17.7 vs 35.7±23.0 months), symptoms (leukocoria in 49.4% vs 62.9%), and outcomes (death in 40.1% vs 13.9%), respectively (P<0.001). In the germline group 66.5% and in non-germline group over 97% of patients had stage Va or higher (ICRB D-E disease). Disease-free survival was 48.6% for germlines cases versus 80.9% for non-germline patients (with mean follow up of 61.9 months, P<0.001). Histopathologically, more invasions to intraocular and extraocular tissues were seen with non-germline tumors of (66% vs 39.8%). Mortality rates in germline cases and non-germline were 40.1% and 13.9%, respectively (P<0.001).
  
  
  CONCLUSIONS
  Despite higher tumor staging in nongermline cases at the time of diagnosis and therefore more aggressive behavior of the tumor, germline cases had a higher rate of mortality during the follow up period.
authors.affiliation: Ocular Oncology and Retina &Vitreous Service, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Fariba
authors.initials: F
authors.lastname: Ghassemi
authors.affiliation: Ocular Oncology and Retina &Vitreous Service, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Hormoz
authors.initials: H
authors.lastname: Chams
authors.affiliation: Department of Epidemiology, Faculty of Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran ; Health Promotion and Injury Prevention Research Centre, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Siamak
authors.initials: S
authors.lastname: Sabour
authors.affiliation: Ocular Oncology and Retina &Vitreous Service, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Reza
authors.initials: R
authors.lastname: Karkhaneh
authors.affiliation: Ocular Oncology and Retina &Vitreous Service, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Farzad
authors.initials: F
authors.lastname: Farzbod
authors.affiliation: Ocular Oncology and Retina &Vitreous Service, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Mehdi
authors.initials: M
authors.lastname: Khodaparast
authors.affiliation: Oncology Service, Mahak Hospital, Pediatric Cancer Research and Hospital, Tehran, Iran.
authors.forename: Parvaneh
authors.initials: P
authors.lastname: Vosough
keywords: Childhood Tumors; Epidemiology; Germline; Non-germline; Retinoblastoma