Retinoblastoma (Rb) is the most common eye cancer in children globally. The disease develops from the back of the eye, and if left untreated, a tumour can develop out of the eye and lead to blindness, metastasis (cancer elsewhere in the body) and death. When a child presents with a tumour like this it is defined as retinoblastoma with extraocular tumor extension (RB-EOE).

A new study by the Retinoblastoma Study Group, facilitated by ICEH researchers, looked at 3435 children with Rb presenting to various treatment centres across the world in 2017. Three hundred and nineteen had RB-EOE and the rest had Rb without an extraocular tumour. The vast majority of children with RB-EOE were from low and lower-middle income countries, with only 8% of those with the condition coming from upper-middle and high-income areas.

Generally, those with RB-EOE took longer to present to treatment centres for diagnosis and were older when they did. 32% of those with RB-EOE developed metastasis compared with only 4% of those without, and critically, 63% died compared to only 6% those without an extraocular tumour.

Within the study, most deaths occurred within 1 year of diagnosis of RB-EOE, underscoring the severity and more aggressive nature of RB-EOE compared with when the cancer is contained within the eye.

The study also looked at what treatment children with RB-EOE received, revealing that 33% had surgery and intravenous chemotherapy together, and 21% had a combination of surgery, intravenous chemotherapy, and radiotherapy (EBRT). In the study, patients who underwent only chemotherapy and surgery had 1.8 times higher chances of death compared with those receiving chemotherapy, surgery, and EBRT, indicating that including EBRT significantly enhances survival rates. The study also showed that those who underwent surgery without chemotherapy first had a 6.5 higher risk of metastasis.

The research draws from comprehensive data that is estimated to have included half of all new Rb cases globally in 2017, and the results highlight differences in outcomes and treatment for RB-EOE across the world. This can inform future decision making and treatment strategies for those with this painful and potentially fatal condition. The authors hope the work can contribute to a standardised approach for the management of RB-EOE.

Publication

Kaliki S, Vempuluru VS, Fabian ID, et al. Retinoblastoma with and without Extraocular Tumor Extension: A Global Comparative Study of 3435 Patients. Ophthalmology Science. 2024;5(2):100637. https://doi.org/10.1016/j.xops.2024.100637