title: Primary intravenous chemotherapy for group D retinoblastoma: a 13-year retrospective analysis.
abstract: BACKGROUND
  Eye salvage rate for group D retinoblastoma using intravenous chemotherapy (IVC) as a primary modality is <50%. To report on 13 years' experience with the use of primary IVC for group D retinoblastoma.
  
  
  METHODS
  A retrospective analysis of 64 group D eyes (52 patients) treated with primary IVC, from 2002 to 2014.
  
  
  RESULTS
  The median age at presentation was 11.0 months (mean: 18.6, range: 0.6-144.0), 35 (67%) patients had bilateral disease, 38 (73%) germline disease and 8 (15%) cases were familial. In addition to IVC, patients received a median number of three treatments (mean: 6, range: 0-24), including thermotherapy/cryotherapy, plaque radiotherapy, intra-ophthalmic artery chemotherapy (IAC) and/or intravitreous chemotherapy. External beam radiotherapy (EBRT) was used in five eyes, all of which were eventually enucleated. In a median follow-up time of 55 months (mean: 64, range: 14-156), 63% of eyes were salvaged. By the Kaplan-Meier survival analysis, globe salvage rate was 83%, 70%, 59% and 45% at 1, 3, 5 and 10 years, respectively. There were no cases of metastatic spread from intraocular retinoblastoma and no deaths. IVC-related adverse events included febrile neutropenia in 21 (40%) patients and anaphylactic reaction to carboplatin in 2 (4%), all conservatively resolved. Of the patients receiving IAC, third and sixth nerve palsies were documented in two (10%) and one (5%) eyes, respectively.
  
  
  CONCLUSIONS
  Primary IVC for group D eyes, with adjuvant treatments as required, was found to be a safe and efficient approach, achieving 63% eye salvage rate, no metastatic spread from intraocular retinoblastoma and no deaths. IAC has now replaced EBRT as a successful salvage treatment.
authors.affiliation: Ocular Oncology Service, Moorfields Eye Hospital NHS Foundation Trust, London, UK.|Retinoblastoma Service, Royal London Hospital, London, UK.
authors.forename: Ido D
authors.initials: ID
authors.lastname: Fabian
authors.affiliation: Ocular Oncology Service, Moorfields Eye Hospital NHS Foundation Trust, London, UK.|Retinoblastoma Service, Royal London Hospital, London, UK.|Department of Ophthalmology, University of Washington, Seattle, Washington, USA.
authors.forename: Andrew W
authors.initials: AW
authors.lastname: Stacey
authors.affiliation: Retinoblastoma Service, Royal London Hospital, London, UK.
authors.forename: Kenneth P
authors.initials: KP
authors.lastname: Johnson
authors.affiliation: Retinoblastoma Service, Royal London Hospital, London, UK.
authors.forename: Zerrin
authors.initials: Z
authors.lastname: Onadim
authors.affiliation: Retinoblastoma Service, Royal London Hospital, London, UK.|Paediatric Oncology Department, Great Ormond Street Hospital, London, UK.
authors.forename: Tanzina
authors.initials: T
authors.lastname: Chowdhury
authors.affiliation: Retinoblastoma Service, Royal London Hospital, London, UK.|Paediatric Oncology Department, Great Ormond Street Hospital, London, UK.
authors.forename: Catriona
authors.initials: C
authors.lastname: Duncan
authors.affiliation: Ocular Oncology Service, Moorfields Eye Hospital NHS Foundation Trust, London, UK.|Retinoblastoma Service, Royal London Hospital, London, UK.
authors.forename: M Ashwin
authors.initials: MA
authors.lastname: Reddy
authors.affiliation: Ocular Oncology Service, Moorfields Eye Hospital NHS Foundation Trust, London, UK.|Retinoblastoma Service, Royal London Hospital, London, UK.|University College London, Institute of Ophthalmology, London, UK.
authors.forename: Mandeep S
authors.initials: MS
authors.lastname: Sagoo
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D017679:Cryotherapy / Q000379:methods; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007261:Infusions, Intra-Arterial; D008297:Male; D019572:Retinal Neoplasms / Q000188:drug therapy*; D012175:Retinoblastoma / Q000188:drug therapy*; D012189:Retrospective Studies; D016879:Salvage Therapy / Q000379:methods; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D005047:Etoposide; D016190:Carboplatin
keywords: Neoplasia