Treatment Outcome of Children with Retinoblastoma in a Tertiary Care Referral Hospital in Indonesia.

Krisna Handayani,Gertjan Kaspers,Saskia Mostert,Pudjo Widjajanto,Braghmandita Indraswari,Wijnanda Kors,Sri Mulatsih,Mei Sitaresmi

doi:10.31557/apjcp.2021.22.5.1613

Abstract

Background:Although survival rates for retinoblastoma (RB) are over 95% in high-income countries, its high mortality rate in low and middle-income countries remains a great concern. Few studies investigated treatment outcome and factors contributing to RB survival in these latter settings. Aims of this study are to determine treatment outcome of Indonesian children diagnosed with RB and to explore factors predictive of treatment outcome. Methods:This study was a retrospective medical records review combined with an illustrative case report. Children newly diagnosed with RB between January 2011 and December 2016 at a tertiary care referral hospital in Indonesia were included. A home visit was conducted to perform an in-depth interview with a mother of two children affected by RB. Results:Of all 61 children with RB, 39% abandoned treatment, 21% died, 20% had progressive or relapsed disease and 20% event-free survival. Progressive or relapsed disease was more common in older (≥ 2 years at diagnosis, 29%) than young (<2 years at diagnosis, 0%) children (P=0.012). Event-free survival estimate at 5 years was higher in young (42%) than older (6%) children (P=0.045). Odds-ratio for event-free survival was 6.9 (95% CI: 1.747 – 27.328, P=0.006) for young versus older children. Other clinical and socio-demographic characteristics had no significant correlation with treatment outcome or event-free survival. The case report elucidated conditions and obstacles that Indonesian families face when their children are diagnosed with RB. Conclusion:Survival of children with RB in Indonesia is much lower compared to high-income and many other low and middle-income countries. Abandonment of treatment is the most common cause of treatment failure. Older age at diagnosis is associated with more progressive or relapsed disease and worse survival. Interventions to improve general public and health-care providers’ awareness, early detection and treatment adherence are required.

Highlights

Retinoblastoma (RB) is the most common intraocular malignancy of childhood
Background: survival rates for retinoblastoma (RB) are over 95% in high-income countries, its high mortality rate in low and middle-income countries remains a great concern
The five year survival rate in high-income countries is over 95%, but often less than 40% in low and middle-income countries

Summary

Introduction

Retinoblastoma (RB) is the most common intraocular malignancy of childhood. Most children are younger than 5 years old at diagnosis, and the average age at diagnosis is 2 years. Whereas the goals of treatment in high-income countries have shifted towards globe or vision salvage and quality of life improvement, low and middle-income countries still focus on saving lives (Cassoux et al, 2017; Fernandes et al, 2017; Gao et al, 2016; Naseripour, 2012; Singh and Daniels, 2016). In the latter countries, the high mortality rate associated with RB is still of great concern (Jain et al, 2018; Naseripour, 2012). Interventions to improve general public and health-care providers’ awareness, early detection and treatment adherence are required

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