title: Long-term outcomes of Group D eyes in bilateral retinoblastoma patients treated with chemoreduction and low-dose IMRT salvage.
abstract: BACKGROUND
  To evaluate outcomes of Group D eyes of bilateral retinoblastoma patients treated with primary chemoreduction and external beam radiation as salvage.
  
  
  METHODS
  Retrospective chart review of patients diagnosed with bilateral retinoblastoma and designated Group D in at least one eye from January 1, 2000 to December 31, 2009. Overall, 62 Group D eyes of 49 patients were included; 13 had bilateral Group D disease. Primary chemoreduction with vincristine, etoposide, and carboplatin with local consolidation was administered, followed by external beam radiation in the form of intensity-modulated radiation therapy (IMRT) as salvage for recurrent tumor. Primary outcome measure was globe salvage.
  
  
  RESULTS
  Of 62 Group D eyes, 7 were enucleated primarily; 55 were treated with systemic chemoreduction, and local therapy. Chemoreduction cured 26 of 55 eyes (47%). Recurrences were found in 29 eyes; 5 underwent enucleation and 24 were treated with IMRT at a dose of 24 Gy (2 eyes) or 36 Gy (22 eyes). Of the 24 irradiated eyes, 19 (79%) were salvaged and 5 required enucleation. Final visual acuity ranged from 20/20 to light perception with 10 eyes having 20/80 or better visual acuity. Average follow-up was 54.2 months.
  
  
  CONCLUSIONS
  Kaplan-Meier estimates of eye survival of Group D eyes in bilateral patients at 12 months is 82% (95% confidence interval [CI] 70.1-89.7%); at 60 months eye survival is estimated to be 68% (95% confidence interval [CI] 55.4-82.8%). Systemic treatment for retinoblastoma demonstrated a high rate of globe preservation with acceptable complications and many eyes retaining functional vision.
authors.affiliation: Doheny Eye Institute, Los Angeles, California 90033, USA. jesse.berrymd@gmail.com
authors.forename: Jesse L
authors.initials: JL
authors.lastname: Berry
authors.forename: Rima
authors.initials: R
authors.lastname: Jubran
authors.forename: Jonathan W
authors.initials: JW
authors.lastname: Kim
authors.forename: Kenneth
authors.initials: K
authors.lastname: Wong
authors.forename: Simon R
authors.initials: SR
authors.lastname: Bababeygy
authors.forename: Hashem
authors.initials: H
authors.lastname: Almarzouki
authors.forename: Thomas C
authors.initials: TC
authors.lastname: Lee
authors.forename: A Linn
authors.initials: AL
authors.lastname: Murphree
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D016190:Carboplatin / Q000008:administration & dosage; D059248:Chemoradiotherapy / Q000379:methods; D002675:Child, Preschool; D005047:Etoposide / Q000008:administration & dosage; D006801:Humans; D007223:Infant; D050397:Radiotherapy, Intensity-Modulated; D019572:Retinal Neoplasms / Q000628:therapy*; D012175:Retinoblastoma / Q000628:therapy*; D012189:Retrospective Studies; D016879:Salvage Therapy / Q000379:methods*; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D005047:Etoposide; D016190:Carboplatin