Management and Outcomes of Retinoblastoma Cases Presenting to Children’s Hospital Westmead, Sydney Between 2008 and 2018

Abstract

This retrospective review reports on the management and outcomes of retinoblastoma in children treated at Children's Hospital Westmead (CHW), Sydney. Results were compared to those of a previous retrospective review of RB cases presenting between 1974 and 2005 at the same centre, which was published in this journal. A retrospective review of all cases of retinoblastoma presenting to the Children's Hospital Westmead Medical between 2008 and 2018 was conducted. 67 patients were included in the study with a mean age at presentation of 23.5-months and 9.2-months for unilateral and bilateral disease respectively. All patients in our cohort were offered genetic testing. The rate of germline RB1 mutation in our cohort was 29% for unilateral disease and 86% for bilateral disease. Mean follow-up period was 48 months. Globe salvage rates in patients with bilateral disease was 57%, compared to the previous study which was 47%. The most common treatment-related ocular complication was strabismus. Our cohort had only one patient develop metastatic disease and one patient who presented with trilateral disease, which was a case of delayed presentation and was the only mortality in the study. Morbidity and mortality rates in our cohort are on par with other tertiary centres internationally. There has been a significant improvement in globe salvage rate with our current management protocol. As intra-arterial chemotherapy is implemented into the treatment regime at CHW, these results will provide a benchmark to ensure that the excellent standards of care and outcomes are maintained.