title: Clinical presentation and survival of retinoblastoma in Indian children.
abstract: OBJECTIVE
  To study the clinical presentation and survival among Indian children with retinoblastoma (RB) and to determine factors predictive of poor outcome.
  
  
  METHODS
  A retrospective review of children newly diagnosed with RB at a tertiary referral centre was undertaken. Demographic and clinical characteristics and treatment outcomes were studied.
  
  
  RESULTS
  A total of 600 patients (unilateral 67.6%, bilateral 32.4%) was studied. 61% was boys. The median age at presentation was 29 months (18 months vs 36 months in bilateral and unilateral cases, respectively, p<0.001). leukocoria was most common (83%), followed by proptosis (17%). Tumours were intraocular in 72.3% and extraocular in 27.7% cases. In the intraocular group, 78% were advanced Group D or E disease. Metastasis to the central nervous system was noted in 15.7% of extraocular cases. A statistically significant difference was seen between intraocular and extraocular groups in the median age (24 months vs 37.5 months, p<0.001) and median lag period (2.5 months vs 7 months, p<0.001). The Kaplan-Meier survival probability was 83%, 73% and 65% at 1 year, 2 years and 5 years, respectively. On univariate analysis, age >2 years (p=0.002), lag period >6 months (p=0.004) and extraocular stage (p<0.001) were associated with poor outcome. On multivariate analysis, extraocular invasion was predictive of low survival (HR 5.04, p<0.001).
  
  
  CONCLUSIONS
  Delayed presentation is a matter of concern. Improving awareness about the early signs and creating facilities for diagnosing and treating RB at the primary and secondary levels of healthcare are required to reduce mortality and morbidity, and lead to improved outcomes that are comparable with the developed nations.
authors.affiliation: Ocular Oncology & Pediatric Ophthalmology Service, Dr Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi, India All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Bhavna
authors.initials: B
authors.lastname: Chawla
authors.affiliation: Ocular Oncology & Pediatric Ophthalmology Service, Dr Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi, India All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Fahmi
authors.initials: F
authors.lastname: Hasan
authors.affiliation: Ocular Oncology & Pediatric Ophthalmology Service, Dr Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi, India All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Rajvardhan
authors.initials: R
authors.lastname: Azad
authors.affiliation: All India Institute of Medical Sciences, New Delhi, India Pediatric Oncology Division, Department of Pediatrics, New Delhi, India.
authors.forename: Rachna
authors.initials: R
authors.lastname: Seth
authors.affiliation: All India Institute of Medical Sciences, New Delhi, India Department of Biostatistics, New Delhi, India.
authors.forename: Ashish Datt
authors.initials: AD
authors.lastname: Upadhyay
authors.affiliation: All India Institute of Medical Sciences, New Delhi, India Department of Radiotherapy, Dr. B.R.A.IRCH, New Delhi, India.
authors.forename: Sushmita
authors.initials: S
authors.lastname: Pathy
authors.affiliation: All India Institute of Medical Sciences, New Delhi, India Department of Biostatistics, New Delhi, India.
authors.forename: R M
authors.initials: RM
authors.lastname: Pandey
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D044466:Asian People / Q000706:statistics & numerical data; D002648:Child; D002675:Child, Preschool; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007194:India / Q000453:epidemiology; D007223:Infant; D008297:Male; D011379:Prognosis; D061766:Proton Therapy; D019572:Retinal Neoplasms / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D012175:Retinoblastoma / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate
chemical_list: D000970:Antineoplastic Agents
keywords: Child health (paediatrics)