Lag time to diagnosis as a predictor of mortality in children with extraocular retinoblastoma: Experience from a developing country

Abstract

Aim To define the prevalence of mortality and identify factors associated with mortality in pediatric patients with extraocular retinoblastoma attending the tertiary hospital in Indonesia. Methods We retrospectively collected medical records from 2013 to 2019 of patients who were diagnosed with extraocular retinoblastoma. Cox proportional hazard regression analysis with 95% confidence interval (CI) was used to evaluate the association of mortality predictors with patient outcomes (Hazard Ratio [HR], 95% CI) with significance set as p < .05. Results Overall, 60 patients were included in this study for a retrospective chart review, with 55% males and 45% females. The median age at diagnosis was 13 (5–24) months. About 60% of the patients did not survive, while 2‐year survival probability was 45%. The overall median survival time was 21.5 (7.25–40.75) months. Predictors of mortality were laterality (unilateral/bilateral): HR 2.15 (95% CI: 1.07–4.28; p = .03), nutritional status: HR 2.65 (95% CI: 1.34–5.25; p = .05), and lag time to diagnosis: HR 3.12 (95% CI: 1.56–6.2; p = .001). Conclusion Laterality, nutritional status and lag time to diagnosis were identified to be mortality predictors in extraocular retinoblastoma. The 2‐year survival for children with extraocular retinoblastoma was 45% with 21 months for median survival.

Lag time, high-risk histopathological features, metastasis, and survival interrelation in retinoblastoma: a perspective from lower-middle income country

“…Other studies have reported that a delayed diagnosis of retinoblastoma resulted in advanced disease upon presentation, with a higher likelihood of high-risk histological characteristics, a lower probability of globe salvage, and the requirement for adjuvant chemotherapy [12,[15][16][17] . In agreement with those previous studies, lag time was also shown to be significantly correlated with an increased mortality in retinoblastoma [18] . Although there were many studies that have already focused on lag time in retinoblastoma, investigations about this important concern in lower-middle income countries, including Indonesia, are limited.…”

Genetic Component of Retinoblastoma in Bukavu, Democratic Republic of Congo

“…Commonly described mutations are nonsense (56%), frameshift (12%), splice site changes (12%) and missense (12%). Exons 8,9,10,11,16,17,18,19,20,21,23 have been reported as frequent mutation carriers [9,10].…”