title: Extensively necrotic retinoblastoma is associated with high-risk prognostic factors.
abstract: BACKGROUND
  Retinoblastoma is the most common malignant intraocular tumor in children. It has been shown that adjuvant therapy following enucleation in patients with high-risk histopathologic features significantly decreases the mortality. We describe the association of extensive necrosis of tumor and intraocular structures with 2 of the major risk factors: optic nerve invasion and choroidal invasion. This may alert the pathologist who makes the observation of extensive necrosis to carefully search for histologic features associated with adverse outcome.
  
  
  OBJECTIVE
  To determine whether extensively necrotic retinoblastoma is associated with high-risk histologic prognostic factors for metastatic disease and patient survival.
  
  
  METHODS
  Retrospective case series. Forty-three eyes of 43 patients with retinoblastoma who underwent enucleation between 1990 and 2001 were evaluated. Medical records, histopathology specimens, pathology reports, and clinical photographs were reviewed. Tumors were designated as exhibiting extensive necrosis if more than 95% of tumor cells and intraocular tissues were necrotic. The main outcome measure was the association of extensive tumor necrosis with 3 high-risk histopathologic features: extraocular extension, optic nerve invasion, or choroidal invasion. Metastatic disease, patient survival, and associations with pathologic findings were also analyzed.
  
  
  RESULTS
  Optic nerve head invasion (P < .001), post-lamina-cribrosal invasion (P < .001), and choroidal invasion by tumor (P = .004) were observed more frequently in eyes with extensive necrosis compared with eyes without extensive necrosis. Two of the 11 patients with extensively necrotic intraocular retinoblastoma died from metastatic disease (P = .06). None of the 32 patients without extensive necrosis developed metastatic disease or died.
  
  
  CONCLUSIONS
  Extensive ocular tissue and tumor necrosis is associated with histologic high-risk prognostic factors for tumor metastasis and mortality.
authors.affiliation: Department of Ophthalmology, Baylor College of Medicine, Houston, Tex, USA.
authors.forename: Eva-Marie
authors.initials: EM
authors.lastname: Chong
authors.forename: Robert E
authors.initials: RE
authors.lastname: Coffee
authors.forename: Murali
authors.initials: M
authors.lastname: Chintagumpala
authors.forename: Richard L
authors.initials: RL
authors.lastname: Hurwitz
authors.forename: Mary Y
authors.initials: MY
authors.lastname: Hurwitz
authors.forename: Patricia
authors.initials: P
authors.lastname: Chévez-Barrios
mesh_terms: D001932:Brain Neoplasms / Q000401:mortality / Q000556:secondary; D017024:Chemotherapy, Adjuvant; D002829:Choroid / Q000473:pathology*; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009336:Necrosis; D009361:Neoplasm Invasiveness; D009900:Optic Nerve / Q000473:pathology*; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D019572:Retinal Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D012175:Retinoblastoma / Q000401:mortality / Q000473:pathology* / Q000556:secondary / Q000601:surgery; D012307:Risk Factors