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

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.

Retinoblastoma in South Asia: A Scoping Review

“…For chemotherapy, Miranda et al used different chemotherapy regimens: Carboplatin alone, Carboplatin + Etoposide, Carboplatin + Vincristine, Carboplatin, Etoposide and Vincristine (VEC), and Methotrexate [13]. The VEC protocol was given for 6 cycles by Handayani et al even after primary enucleation of intraocular disease [17]. For those with regional extension, the Retinoblastoma 2002 Grabowski-Abramson (GA) II/III Protocol was followed where after primary enucleation was done, 105 weeks of vincristine, methotrexate, doxorubicin and cyclophosphamide were For chemotherapy, intravenous vincristine, etoposide and carboplatin were given 3 to 4 weeks for six cycles for mass reduction [3].…”

“…Patients who were more than 5 years old were high at 6 (13%) in one series [15]. Median delay of consultation in a series was 12 months (0-60) [17]. Delay in consultation was primarily from socioeconomic reasons followed by lack of awareness of the disease and need for early intervention [12].…”

“…Seventy-nine percent had unilateral disease. Presence of family history was low at 0.6% attributed to the low over-all survival rate of retinoblastoma patients given [17]. For metastatic disease, GA IV Protocol was used instead where cytarabine and etoposide were added to GA II/III Protocol [17].…”

“…Presence of family history was low at 0.6% attributed to the low over-all survival rate of retinoblastoma patients given [17]. For metastatic disease, GA IV Protocol was used instead where cytarabine and etoposide were added to GA II/III Protocol [17]. The most common side-effect noted from chemotherapy was anemia found in 70% of the patients [15].…”

“…Only one article has data on survival rate which was at 20% [17]. Treatment refusal was present in 16% which was attributed to lack of parents' understanding of the disease.…”