Retinoblastoma – Lessons Learned about Patterns of Care and Contributory Factors from 5 Years’ Experience in a Tertiary Care Center in Eastern India

Abstract

Objectives: Retinoblastoma is the most common intraocular malignancy afflicting children worldwide. Even though there are enough data about the epidemiology of retinoblastoma in western population, there are only few reports from developing countries like India. In this retrospective study, we aimed to describe the epidemiological patterns, survival characteristics and prognostic factors of retinoblastoma.
 Materials and Methods: From medical records, we retrospectively analyzed the data of 68 children diagnosed in our hospital between January 2013 and December 2017 as having retinoblastoma. Data on sex, religion, laterality, age at diagnosis, presenting signs, family history, lag time for treatment, cause of such lag time and spread of tumor, treatment mode, and survival time were collected.
 Results: The median age of onset was 22 ± 19.73 months (range 2-92 months).The median patient age of onset of the unilateral cases was 23 ± 20.6 months, and that of the bilateral cases was 21 ±16.2 months. The median overall survival was 28.1±2.2 months. For unilateral cases, it was 30.1±2.5 months and for bilateral cases it was 19.7±2.9 months. The overall progression free survival (PFS) was 22.2±2.3 months. For unilateral cases, it was 24.18±2.7 months and for bilateral cases it was 13.9±2.9 months.
 4 cases of familial retinoblastoma were reported. Among the 13 bilateral cases, 3 were found to have pinealoblastoma too. On Cox regression analysis, age of onset below 36 months, diagnostic delay of less than 5 months and delay of treatment initiation (after diagnosis) less than 2 months were found to have significant effect on OS. The former two were found to have significant effect on PFS but not the latter (p<0.05 and HR>1).
 Conclusions: Almost 81% of patients presented at an advanced stage of the disease, the reason being accounted by diagnostic and therapeutic delay by virtue of a number of causes, the major one being eluded by apparently nonviolent yet ineffective alternative medicine practices. In spite of following the institutional protocols which are at par to the international guidelines, analysis shows much poorer survival in this study compared to those of developed countries. The cause might be such late presentation of the cases in already advanced stages of the disease.

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

“…24 Other studies have shown an association, although it was not significant between the lag time to diagnosis and death, which had a lower cut-off, in 3 months (p = .96), 5 months with HR 7.7 (.129-46.13), and 6 months (95% CI: OR .71-1.51). 15 The lag time to therapy in this study has a median value of 1 month (0-7.75). This is consistent with a previous study in India that showed the lag time to therapy interval ranged from days to 12 months, with a median value of 1 month.…”

“…In a study in India, the onset was 22 ± 19.7 months. 15 Research in Brazil showed a median of 18 months (1-58 months). 17 The onset varied depending on the dominance of the study subjects having hereditary or nonhereditary retinoblastoma.…”

“…This finding is in accordance with previ-ous study in India which stated that the most frequent symptoms were leukocoria, proptosis, and blurred vision (44.1, 26.5, and 13.2%). 15 Endophytic (to the vitreous) or exophytic (to the choroid) spread can cause calcification, which gives rise to white patches that cause leukocoria symptoms and these symptoms are easily observed. 18 The most common extraocular retinoblastoma stage in this study was stage IV, with 63.3%, compared to stage III in 36.7% of subjects.…”

“…A retrospective study in India reported the delay was only 5 months (1-36). 15 Research conducted in Brazil showed the median lag time to diagnosis was 5 months (0-35). 17 This difference occurred because the Brazilian study was conducted with 72% of patients from urban areas, and with a parental education level above senior high school, whereas in this study, the research subjects were geographically scattered, and the level of education was relatively the same between junior high school or lower with high school or higher (28 vs. 32).…”

“…This is consistent with a previous study in India that showed the lag time to therapy interval ranged from days to 12 months, with a median value of 1 month. 15 A median interval of only 1 month means that the patient's family understands the explanation given by the doctor at Dr. Sardjito General Hospital regarding the importance of therapy in the case of extraocular retinoblastoma. The delay in therapy for patients with retinoblastoma in Taiwan involved cultural factors, rejection of enucleation, and alternative medicine.…”