Clinical presentation and outcome of children with Retinoblastoma managed at Uganda Cancer Institute

Abstract

Retinoblastoma is the most common primary malignant tumour of childhood and the worst outcomes of the disease are in developing countries. Retinoblastoma is associated with high mortality and morbidity and thus requires timely diagnosis, treatment and follow-up. Clinical extraocular disease has been reported to be the commonest and usually fatal manifestation of retinoblastoma in developing countries. The number of children with retinoblastoma received and treated at Uganda cancer institute (UCI) has increased steadily over the last 12 years. However, no documented study has been conducted to assess their clinical presentation pattern, outcome and predictors of outcome in the last 12 years at Uganda Cancer institute, the national referral oncology facility. Aim: To determine the clinical presentation, outcome and predictors of survival in children with retinoblastoma managed at the paediatric oncology unit of Uganda Cancer Institute. Methods and Materials: This was a retrospective cross-sectional study of children with retinoblastoma admitted and treated at the Paediatric oncology unit of Uganda Cancer Institute from 1st January 2009 to 28th February 2020. IRB approval and administrative clearance to conduct research was obtained from UCI. Retinoblastoma patients’ file numbers were obtained from the electronic database and charts/files that met then inclusion criteria were selected. The patients` demographic factors, history, clinical presentation, examination findings, investigations, treatment modalities used and outcomes were captured using a pre-tested data extraction tool. Using telephone contacts of Patients` caretakers in the charts, verbal consent was obtained and missing information was obtained via telephone interview using a pre-tested questionnaire. Data was entered into the computer using Epi data 4.2 by double entry method and analysed using STATA 15.1/MP. Results: A total 90 Retinoblastoma patients were studied, with an overall mean age at first sign of 25.5 months (SD 26.0) and mean age at first UCI presentation of 36.7 months (SD 25.5). There were more males than females with Male to Female Ratio of 1.37:1. A bigger percentage 36.7% (n=33) came from the central region of the country. Children from neighbouring countries constituted 13.3% (n=12). The commonest presenting symptom was leukocoria reported in 85.6% (n=77) children, followed by Eye Reddening in 64.4% (n=58) and eye swelling in 63.3%(n=57). Majority, 49 (54.4%) had prior retinoblastoma treatment prior to UCI admission with significant number (46.7% (n=42) with at least one an enucleation. Sixty-five (72.3%) presented with Unilateral Rb and 25 (27.8%) with bilateral disease. Near all children (98.7%) had worst affected eye with IIRC Group D and worse. The median survival time in months for children with Rb children was 26.5 (8.6 –61.0) and overall survival was 15% (1.1% -45.1). There were 38.9% death and 22% lost follow-ups. The factors that were statistically significant predictors of mortality at multivariate analysis were, shorter follow-up duration, features metastatic spread, history of eye swelling, and bilateral enucleation. Conclusions and recommendations: There was a high mortality rate of children with retinoblastoma and majority were referred from eye units to UCI with advanced disease for further treatment. For better outcomes, increased awareness and screening for retinoblastoma is highly needed for early detection and timely treatment. In addition, retinoblastoma management requires a multi-disciplinary team that includes Paediatric ophthalmologists, Paediatric oncologists, ocular oncologists nurses among others.