Management of unilateral retinoblastoma with buphthalmia

Purpose Locally advanced unilateral retinoblastoma (RB) not amenable to conservative treatments is treated with enucleation. Surgery is challenging in presence of buphthalmia, exophthalmia or severe periocular inflammation, carrying a risk for globe rupture with tumour dissemination. The aim of this study was to report the long-term results of neoadjuvant chemotherapy (CT) in this setting. Methods Retrospective case series of unilateral group E RB patients without extraocular disease receiving pre-enucleation CT (etoposide-carboplatin) in a single centre between 1999 and 2011. Clinical, radiological and histopathological data were collected. Outcome measures were: surgical complications, pathological risk factors (PRF) for metastatic dissemination, systemic side effects, metastatic dissemination, survival. Results 21 patients, median age 9 months (range 1.5–48) were included: 16 with clinical buphthalmia, 2 with orbital inflammation and 3 with uncontrollable ocular hypertension. MRI confirmed the clinical findings and ruled out extraocular disease in all cases. Enucleation was performed after 1 course of neoadjuvant CT in 16/21 (77%), the remaining 5 patients needing two. No surgical complications occurred. Pathological examination showed complete resection in all cases. PRF for were present in 7/21 (33%) eyes. Irrespectively, all children completed a 4-cycle CT regimen, as CT could mask PRF. Two children required hospitalisation for complicated aplasia, no other systemic side effects were reported. With a median follow-up time of 8 years (range 4–12), no metastatic dissemination occurred. Survival is 100%. Conclusions Locally advanced unilateral RB with buphthalmia can be successfully treated with neoadjuvant CT prior to enucleation to make the surgery safer.