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Longitudinal Evaluation of Functioning in Children With Retinoblastoma


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In a single-institution study reported in the Journal of Clinical Oncology, Willard et al found that improvements in cognitive and adaptive function were observed between ages 5 and 10 years in many children with retinoblastoma, with scores by age 10 generally being within normal limits. Poorer earlier outcomes were associated with Black race, unilateral disease, treatment including enucleation, and treatment with enucleation alone.

A prior report on the cohort showed declines in function between diagnosis and age 5.

Study Details

The study involved 98 patients at St. Jude Children’s Research Hospital who completed psychological assessments as part of enrollment in an institutional treatment protocol; of these, 73 completed an additional assessment at age 10. Assessments were completed at the ages of 6 months and 1, 2, 3, 5, and 10 years. The treatment protocol consisted of strata, in which strata A and B received chemotherapy, stratum C-high risk (C-high) received enucleation followed by chemotherapy, and stratum C-low risk (C-low) received enucleation alone. Adaptive functioning was assessed by the Vineland Adaptive Behavior Scales (completed by caregivers at all time points), with three subscales (Daily Living Skills, Communication, and Socialization) used to derive an overall Adaptive Behavior Composite score. Developmental or cognitive functioning was assessed by the Mullen Scales of Early Learning for patients aged ≤ 5 years, and the Wechsler Abbreviated Scales of Intelligence, second edition (yielding an estimated intelligence quotient [EIQ]) was used for patients aged 10 years. A group of 10 patients with 13q-deletion syndrome, associated with intellectual disability, was analyzed separately.

Key Findings

KEY POINTS

  • Overall, longitudinal trajectories showed a significant change point at age 5, with functioning declining from diagnosis through age 5 and then increasing from age 5 to age 10.
  • Black patients showed the greatest decline in function through age 5, with the decline being significantly greater vs White patients, who showed the smallest decline. After age 5, improvement was significant among Black patients and marginal among White patients.
  • The cognitive functioning trajectory was affected by laterality and history of enucleation.

Overall, longitudinal trajectories showed a significant change point at age 5, with functioning declining from diagnosis through age 5 and then increasing from age 5 to age 10.

For adaptive functioning, the mean Adaptive Behavior Composite score for all patients was in the average range and consistent with the normative mean at age 10; patients in stratum A (chemotherapy) had a mean score above the normative mean (better functioning vs normative mean; P = .04). The pattern of decline in function through age 5 and increase in function between 5 and 10 years of age was observed in all treatment strata.

The adaptive functioning trajectory was affected by race. Black patients showed the greatest decline in function through age 5, with the decline being significantly greater vs White patients (P = .02), who showed the smallest decline. After age 5, improvement was significant among Black patients and marginal among White patients (P = .02 for difference). 

For cognitive functioning, the mean EIQ for all patients was within the average range, but significantly below the normative mean (P < .05). There were no significant differences by stratum, although patients in stratum C-low (enucleation only) had the lowest mean score (P < .01 vs normative mean). The pattern of increase in functioning between 5 and 10 years of age was observed among all patients; analysis by strata showed improvement in all, with a significant improvement in stratum C-low. 

The cognitive functioning trajectory was affected by laterality and history of enucleation. Patients with unilateral disease vs bilateral disease showed a greater decline through age 5 (P < .01 for difference). Both groups improved in functioning after age 5, but the slope of improvement was significant only for patients with unilateral disease (P = .01). Patients who did not require surgery showed minimal decline through 5 years vs those undergoing enucleation (P < .001; P = .003 for difference).

Patients with 13q-deletion syndrome demonstrated very low functioning, but few analyses were significant due to the small sample size.

The investigators concluded, “The results generally indicate that previously demonstrated declines in functioning from diagnosis through age 5 improve by age 10. However, these early declines, as well as the continuous difficulties observed in patients treated with enucleation only, suggest the need for early intervention services for young patients with retinoblastoma. Continuous monitoring of the psychological functioning of patients with retinoblastoma; increased awareness of risk factors such as unilateral disease, enucleation, race, and surgery-only treatment plans; and referral to Early Intervention for all patients are indicated.”

Victoria W. Willard, PhD, of the Department of Psychology, St. Jude Children’s Research Hospital, is the corresponding author for the Journal of Clinical Oncology article.

Disclosure: The study was supported by a National Institutes of Health grant and by American Lebanese Syrian Associated Charities. For full disclosures of the study authors, visit ascopubs.org.

The content in this post has not been reviewed by the American Society of Clinical Oncology, Inc. (ASCO®) and does not necessarily reflect the ideas and opinions of ASCO®.
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