Functional decline in children with retinoblastoma greater than expected

1. A significant decline in developmental functioning was seen in children treated for retinoblastoma after age 5 years. 

2. The greatest decline in cognitive functioning seen in children treated with enucleation only. 

Evidence Rating Level: 2 (Good) 

Study Rundown: Previous studies have suggested that survivors of retinoblastoma demonstrate above average cognitive function, while more recent reports have suggested a wider variation in development than previously recognized. The purpose of this study was to evaluate the early developmental trajectory of these patients from diagnosis and treatment to age 5 years.

At the conclusion of this study, the authors found that, while patients’ functional scores were comparable to the norm at the start of the study, there was a significant decline over time with low average scores at age 5 years. Parent-completed reports of adaptive functioning were greater than the mean at 6 months but average at age 5 years. A subset analysis of patients with 13q deletions showed decreased function at baseline but no decline over time. Patients who had undergone enucleation experienced the greatest functional decline. Based on these results, the authors suggest that early cognitive development must be continually reassessed children treated for retinoblastoma before 5 years old. It should be noted that the impact of early assessments on later outcomes is debatable, and that long-term outcomes are based on the limited number of patients who completed all assessments.

Click to read the study in JCO

Relevant Reading: Long-term follow-up of children with retinoblastoma

In-Depth [prospective cohort]: A total of 94 patients diagnosed and treated for retinoblastoma when less than 5 years old were included into analysis for this study. Serial assessments with the Mullen Scales of Early Learning were done at time of study entry, age 6 months and 1, 2, 3 and 5 years. Vineland Scales of Adaptive Behavior were completed by parents. At baseline, the mean early assessment was similar to the normative mean (t(46) = -1.86, p = 0.069). By age 5 years, the mean score was significantly less than the mean (t(32) = -6.07, p < 0.001). Parent report of adaptive functioning was greater than the mean at 6 months but with no difference at age 5 years, with significant decline over time. Patients with 13q mutations were analyzed separately, and demonstrated decreased function at baseline but with no decline over time.

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