title: Management of advanced retinoblastoma with intravenous chemotherapy then intra-arterial chemotherapy as alternative to enucleation.
abstract: OBJECTIVE
  To determine the efficacy of primary intravenous chemotherapy (IVC) plus secondary intraarterial chemotherapy (IAC) for patients with advanced retinoblastoma.
  
  
  METHODS
  Retrospective, nonrandomized interventional case series of 14 patients with retinoblastoma managed with primary systemic IVC (vincristine, etoposide, and carboplatin for 6 cycles) followed by secondary IAC (melphalan for 1-6 cycles).
  
  
  RESULTS
  Fourteen patients with advanced retinoblastoma classified by the International Classification of Retinoblastoma as Group D (n = 6, 43%) or Group E (n = 8, 57%) were treated with IVC as primary treatment and subsequent secondary IAC as rescue or consolidation therapy. The IAC was given for recurrent retinoblastoma and/or subretinal/vitreous seeds in 13 eyes (93%) and for persistent viable retinoblastoma in 1 eye (7%). Enucleation was the alternative option. The mean interval between IVC completion and IAC start was 40 weeks (median, 11 weeks; range, 2-170 weeks) and the mean number of IAC cycles was 3 (median, 3; range, 1-6). After primary IVC plus secondary IAC, globe salvage was achieved in 8 patients (57%) at mean 2-year follow-up. There was no evidence of retinoblastoma metastasis or death and no sign of second cancer or life-threatening complication.
  
  
  CONCLUSIONS
  For advanced retinoblastoma (Groups D and E) in which enucleation is the alternative option, primary systemic IVC followed by secondary focal IAC provides globe salvage in 57% of the eyes and with no metastatic event.
authors.affiliation: *Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania; †Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia; ‡Department of Ophthalmology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; §Division of Pediatric Hematology/Oncology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; ¶Division of Pediatric Hematology/Oncology, A.I. duPont Hospital for Children, Thomas Jefferson University, Wilmington, Delaware; and **Division of Neurovascular and Endovascular Surgery, Neurosurgery Department, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Swathi
authors.initials: S
authors.lastname: Kaliki
authors.forename: Saad
authors.initials: S
authors.lastname: Al-Dahmash
authors.forename: Duangnate
authors.initials: D
authors.lastname: Rojanaporn
authors.forename: Ann
authors.initials: A
authors.lastname: Leahey
authors.forename: Gregory
authors.initials: G
authors.lastname: Griffin
authors.forename: Pascal
authors.initials: P
authors.lastname: Jabbour
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000627:therapeutic use; D002675:Child, Preschool; D005047:Etoposide / Q000627:therapeutic use; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007223:Infant; D007261:Infusions, Intra-Arterial; D007262:Infusions, Intravenous; D008297:Male; D008558:Melphalan / Q000627:therapeutic use*; D019572:Retinal Neoplasms / Q000188:drug therapy* / Q000473:pathology; D012175:Retinoblastoma / Q000188:drug therapy* / Q000473:pathology; D012189:Retrospective Studies; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D018906:Antineoplastic Agents, Alkylating; D014750:Vincristine; D005047:Etoposide; D016190:Carboplatin; D008558:Melphalan