title: Outcomes of a two-drug chemotherapy regimen for intraocular retinoblastoma.
abstract: BACKGROUND
  Retinoblastoma is the most common intraocular tumor of childhood. Vision salvage rates in advanced cases are less than ideal, and the optimal treatment for intraocular retinoblastoma has not been established. We report the results of an institutional retinoblastoma treatment trial to determine the vision salvage rates and toxicity of a regimen combining carboplatin and etoposide with focal retinal therapy.
  
  
  METHODS
  Twenty-nine patients diagnosed with retinoblastoma in 48 eyes were treated between 1992 and 2004 with at least six cycles of carboplatin and etoposide combined with focal retinal therapy.
  
  
  RESULTS
  The response rate of eyes after six cycles of chemotherapy was 85.4%. Twenty-two eyes were enucleated, but only seven eyes received EBRT. The vision salvage rate without EBRT was 82.6% for eyes with Reese-Ellsworth (R-E) groups I-IV tumors and 20% for eyes with R-E group V tumors. The vision salvage rate without EBRT for eyes with Murphree groups A and B tumors was 77.3% but was only 26.9% for eyes with groups C and D tumors. Acute side effects were minimal.
  
  
  CONCLUSIONS
  The combination of carboplatin and etoposide with focal therapy is a well-tolerated regimen that has acceptable vision salvage rates for R-E groups I-IV and Murphree groups A and B retinoblastoma. This combination avoids the use of EBRT and the toxicity of additional chemotherapy agents. However, patients with R-E group V and Murphree groups C and D retinoblastoma have poorer outcomes and require more intensive therapy.
authors.affiliation: Division of Hematology/Oncology/Transplantation, Children's Memorial Hospital, Chicago, Illinois, USA.
authors.forename: Peter E
authors.initials: PE
authors.lastname: Zage
authors.forename: Aaron J
authors.initials: AJ
authors.lastname: Reitman
authors.forename: Roopa
authors.initials: R
authors.lastname: Seshadri
authors.forename: Joanna L
authors.initials: JL
authors.lastname: Weinstein
authors.forename: Marilyn B
authors.initials: MB
authors.lastname: Mets
authors.forename: Janice Lasky
authors.initials: JL
authors.lastname: Zeid
authors.forename: Mark J
authors.initials: MJ
authors.lastname: Greenwald
authors.forename: Lewis C
authors.initials: LC
authors.lastname: Strauss
authors.forename: Stewart
authors.initials: S
authors.lastname: Goldman
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D001766:Blindness / Q000517:prevention & control; D016190:Carboplatin / Q000008:administration & dosage / Q000009:adverse effects; D002675:Child, Preschool; D003131:Combined Modality Therapy; D017679:Cryotherapy; D018450:Disease Progression; D005047:Etoposide / Q000008:administration & dosage / Q000009:adverse effects; D015353:Eye Enucleation; D006801:Humans; D006979:Hyperthermia, Induced; D007223:Infant; D007231:Infant, Newborn; D053685:Laser Therapy; D009364:Neoplasm Recurrence, Local; D009378:Neoplasms, Multiple Primary / Q000188:drug therapy* / Q000601:surgery; D011446:Prospective Studies; D019572:Retinal Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D012175:Retinoblastoma / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome; D014792:Visual Acuity
chemical_list: D005047:Etoposide; D016190:Carboplatin