title: Retinoblastoma: ten years experience at Kanti Children's Hospital.
abstract: BACKGROUND
  Retinoblastoma is a malignant tumor of the embryonic neural retina. It is the most common intraocular tumor of childhood. It is a disease of early childhood with 80% cases diagnosed before the age of 4 years. The median age at diagnosis is 2 years. Bilateral cases are diagnosed earlier (median at 12 months and unilateral median age at 24 months). The incidence of retinoblastoma is approximately 1 in 15000-18000 live births in developed countries. The aim of the research is to study the clinical pathological features, treatment and survival of children with retinoblastoma over ten years.
  
  
  METHODS
  A retrospective hospital based study was conducted from March 1998 to February 2008. Gender, age, clinical presentation, pathology reports, treatment, follow-up and outcome were recorded at time of diagnosis.
  
  
  RESULTS
  A total of 42 children with retinoblastoma (85.7 % unilateral, 14.3% bilateral), with 50% having optic nerve involvement were studied. The most common presenting signs were extraoccular (28.6%), proptosis (23.8%), leukoria (23.8%), phthisis bulbi (16.7%), and strabismus (7.1%). The age at presentation ranged from 6-120 months with mean age of 46.6 months. The male to female ratio was 1.1:1.Two-thirds presented between 2-5 yrs, followed by 12-24 months (23.8%). Majority of children had poorly differentiated retinoblastoma (62 %), followed by well differentiated (28.6%), and moderately differentiated (9.5%). CEV based protocol used to treat, and over-all 10 yrs survival was 23.8 %, death 19 %, and lost to follow-up or left against medical advice 57.2%.
  
  
  CONCLUSIONS
  Despite severe resource limitation, pediatric oncology unit has been successfully treating retinoblastoma with the success rate of 23.8%.
authors.affiliation: Kanti Children's Hospital, Kathmandu, Nepal.
authors.forename: Kailash Prasad
authors.initials: KP
authors.lastname: Sah
authors.affiliation: Tilganga Eye Hospital, Kathmandu, Nepal.
authors.forename: Rohit
authors.initials: R
authors.lastname: Saiju
authors.affiliation: Kanti Children's Hospital, Kathmandu, Nepal.
authors.forename: Pankaj
authors.initials: P
authors.lastname: Roy
authors.affiliation: Kanti Children's Hospital, Kathmandu, Nepal.
authors.forename: Sonali
authors.initials: S
authors.lastname: Kafle
mesh_terms: D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D057210:Delayed Diagnosis; D005260:Female; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D019572:Retinal Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D012175:Retinoblastoma / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D012189:Retrospective Studies; D017678:Sex Distribution; D016896:Treatment Outcome