title: Superselective intra-arterial chemotherapy in the primary management of advanced intra-ocular retinoblastoma: first 4-year experience from a single institution in Turkey.
abstract: OBJECTIVE
  To report our 4-year experience in Turkey, with advanced intra-ocular retinoblastoma managed primarily with intra-arterial chemotherapy (IAC).
  
  
  METHODS
  From October 2011 to September 2015, 26 group D eyes of 24 treatment-naïve retinoblastoma patients managed primarily with IAC were evaluated in this prospective study.
  
  
  RESULTS
  Of 76 procedures, ophthalmic artery cannulation failed in two patients with unilateral involvement. In the remaining 22 patients (24 eyes), the mean age at diagnosis was 18 months (range, 6-55 months). Each eye received a mean of 3 IAC sessions/eye (range, 2-5 sessions). After a median follow-up of 29 months (range, 6-55 months), complete regression of the main tumour was achieved in 23 of 24 eyes. One eye with partial regression required enucleation due to ciliary body involvement by the tumour. Overall, 16 eyes (66.6%) were salvaged with primary IAC with or without additional local treatments, and eight (33.3%) required enucleation. The main IAC-related periocular complications included transient eyelid oedema (n = 13), ptosis (n = 6) and forehead hyperpigmentation (n = 3), each resolving in 2 weeks to 4 months. Intra-ocular complications included chorioretinal atrophy (n = 9), newly noted retinal detachment (n = 5) and vitreous haemorrhage (n = 1). Kaplan-Meier eye estimates of enucleation-free survival rates were 83.3% (95% CI, 68.4-98.1%), 69.1% (95% CI, 49.8-88.3%) and 62.9 (95% CI, 41.9-83.8%) at 6 months, 1 and 2 years, respectively, and stable thereafter.
  
  
  CONCLUSIONS
  Our first 4-year experience in Turkey showed that enucleation or external-beam radiotherapy could be avoided in two-thirds of eyes with advanced intra-ocular retinoblastoma managed primarily with IAC.
authors.affiliation: Istanbul Faculty of Medicine, Department of Ophthalmology, Ocular Oncology Service, Istanbul University, Istanbul, Turkey. sbtuncer@yahoo.com.
authors.forename: Samuray
authors.initials: S
authors.lastname: Tuncer
authors.affiliation: Istanbul Faculty of Medicine, Department of Radiology, Division of Interventional Neuroradiology, Istanbul University, Istanbul, Turkey.
authors.forename: Serra
authors.initials: S
authors.lastname: Sencer
authors.affiliation: Cerrahpasa Medical Faculty and Oncology Institute, Division of Pediatric Hematology - Oncology, Istanbul University, Istanbul, Turkey.
authors.forename: Rejin
authors.initials: R
authors.lastname: Kebudi
authors.affiliation: Istanbul Faculty of Medicine, Department of Ophthalmology, Ocular Oncology Service, Istanbul University, Istanbul, Turkey.
authors.forename: Burak
authors.initials: B
authors.lastname: Tanyıldız
authors.affiliation: Istanbul Faculty of Medicine, Department of Ophthalmology, Ocular Oncology Service, Istanbul University, Istanbul, Turkey.
authors.forename: Zafer
authors.initials: Z
authors.lastname: Cebeci
authors.affiliation: Istanbul Faculty of Medicine, Department of Radiology, Division of Interventional Neuroradiology, Istanbul University, Istanbul, Turkey.
authors.forename: Kubilay
authors.initials: K
authors.lastname: Aydın
mesh_terms: D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage*; D002675:Child, Preschool; D005260:Female; D005471:Fluoroscopy; D006801:Humans; D007223:Infant; D007261:Infusions, Intra-Arterial; D008297:Male; D008558:Melphalan / Q000008:administration & dosage*; D009880:Ophthalmic Artery; D011446:Prospective Studies; D019572:Retinal Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D012175:Retinoblastoma / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome; D014421:Turkey
chemical_list: D018906:Antineoplastic Agents, Alkylating; D008558:Melphalan
keywords: chemosurgery; intra-arterial chemotherapy; primary therapy; retinoblastoma; superselective; superselective intra-arterial chemotherapy