Superselective intra-arterial chemotherapy in the primary management of advanced intra-ocular retinoblastoma: first 4-year experience from a single institution in Turkey.

Purpose 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-naive 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. Conclusion 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.