title: A histopathologic analysis of eyes primarily enucleated for advanced intraocular retinoblastoma from a developing country.
abstract: BACKGROUND
  In eyes enucleated for retinoblastoma, presence of histopathologic high-risk factors is associated with a higher risk of local recurrence and systemic metastasis.
  
  
  OBJECTIVE
  To evaluate histopathologic features in children with retinoblastoma in our population and establish relationship between age, tumor differentiation, and high-risk features.
  
  
  METHODS
  Retrospective histopathologic analysis of 609 consecutively enucleated eyes for advanced intraocular retinoblastoma during a 10-year period. A nonparametric test was used to establish relationship between age, differentiation, and high-risk features.
  
  
  RESULTS
  Poorly differentiated retinoblastoma presented in 80.3% and well-differentiated in 19.7% of eyes. Well-differentiated tumors presented earlier (median 1.2 years) than poorly differentiated tumors (median 2.5 years) (P < .001). One hundred fourteen eyes (18.7%) had 1 and 138 (22.7%) had at least 2 high-risk histopathologic factors. Invasion of anterior chamber was found in 10.0%, iris in 10.7%, ciliary body in 6.7%, sclera in 13.7%, massive choroid in 24.6%, postlaminar optic nerve in 16.1%, resected margin of the optic nerve in 7.4%, and extrascleral tissue in 4.1% of eyes. Extensive necrosis was seen in 31.0% of eyes. Poorly differentiated tumors were significantly associated with presence of more than 1 high-risk histopathologic feature (P < .001) and extensive necrosis (P < .001).
  
  
  CONCLUSIONS
  Poorly differentiated tumors present at a later age and are associated with presence of multiple high-risk factors and extensive necrosis. In our population, high-risk histopathologic factors are present in a significant number of eyes. Because we have included only primarily enucleated eyes, this could truly represent the distribution of high-risk histopathologic factors in children with retinoblastoma.
authors.affiliation: Ocular Pathology Services, Dr R. P. Centre for Ophthalmic Sciences, All India Institute of Ophthalmic Sciences, Ansari Nagar, New Delhi, India.
authors.forename: Seema
authors.initials: S
authors.lastname: Kashyap
authors.forename: Sumita
authors.initials: S
authors.lastname: Sethi
authors.forename: Rachna
authors.initials: R
authors.lastname: Meel
authors.forename: Neelam
authors.initials: N
authors.lastname: Pushker
authors.forename: Seema
authors.initials: S
authors.lastname: Sen
authors.forename: Mandeep Singh
authors.initials: MS
authors.lastname: Bajaj
authors.forename: Mahesh
authors.initials: M
authors.lastname: Chandra
authors.forename: Supriyo
authors.initials: S
authors.lastname: Ghose
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002829:Choroid / Q000473:pathology; D003906:Developing Countries; D005123:Eye / Q000473:pathology*; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009900:Optic Nerve / Q000473:pathology; D019572:Retinal Neoplasms / Q000473:pathology* / Q000601:surgery; D012175:Retinoblastoma / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D055815:Young Adult