title: Postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin for the treatment of high-risk retinoblastoma.
abstract: BACKGROUND
  Analysis of 52 eyes with high-risk retinoblastoma managed with postenucleation adjuvant chemotherapy using vincristine sulfate, etoposide phosphate, and carboplatin showed no evidence of systemic metastasis in any case during a mean (range) follow-up of 66 (12-202) months.
  
  
  OBJECTIVE
  To determine the efficacy of postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin in the prevention of metastasis for patients with high-risk retinoblastoma.
  
  
  METHODS
  Retrospective, nonrandomized, interventional case series of 52 eyes in 51 patients with high-risk retinoblastoma consisting of tumor invasion into the anterior segment, posterior uvea 3 mm or greater, postlaminar optic nerve, or any combination of posterior uvea and optic nerve involvement.
  
  
  RESULTS
  Of 51 consecutive patients with high-risk retinoblastoma, there were 30 males (59%) and 21 females (41%), with a median age of 28 months at diagnosis. All 52 eyes were classified as group E. The main histopathologic risk factors included anterior segment invasion (7 [13%]), isolated massive posterior uveal invasion of 3 mm or greater (6 [12%]), isolated postlaminar optic nerve invasion (15 [29%]), or any posterior uveal invasion with any optic nerve involvement (24 [46%]). There was additional invasion into the sclera (3 [6%]) and extrascleral structures, including the orbit (1 [2%]). A single histopathologic high-risk factor was present in 32 eyes (62%), whereas 20 eyes (38%) manifested 2 or more high-risk characteristics. Based on previously published series, untreated high-risk retinoblastoma carries at least a 24% risk for metastatic disease. In the present series, using vincristine, etoposide, and carboplatin in all cases, there was no metastasis during a mean follow-up of 66 months (median [range], 55 [12-202] months).
  
  
  CONCLUSIONS
  Retinoblastoma with invasion into the postlaminar optic nerve and/or posterior uvea is at high risk for metastasis and death. In this study, postenucleation chemotherapy using vincristine, etoposide, and carboplatin was effective in preventing metastasis in every case (100%).
authors.affiliation: Ocular Oncology Service, Wills Eye Institute, Philadelphia, PA 19107, USA.
authors.forename: Swathi
authors.initials: S
authors.lastname: Kaliki
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Sanket U
authors.initials: SU
authors.lastname: Shah
authors.forename: Ralph C
authors.initials: RC
authors.lastname: Eagle
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
authors.forename: Ann
authors.initials: A
authors.lastname: Leahey
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D005047:Etoposide / Q000627:therapeutic use; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009361:Neoplasm Invasiveness; D019574:Optic Nerve Neoplasms / Q000401:mortality / Q000517:prevention & control* / Q000556:secondary; D011182:Postoperative Care; D019572:Retinal Neoplasms / Q000401:mortality / Q000473:pathology / Q000517:prevention & control*; D012175:Retinoblastoma / Q000401:mortality / Q000517:prevention & control* / Q000556:secondary; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D014604:Uveal Neoplasms / Q000401:mortality / Q000517:prevention & control* / Q000556:secondary; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D014750:Vincristine; D005047:Etoposide; D016190:Carboplatin