Affiliation:
1. University of Vermont Children's Hospital Burlington Vermont USA
2. St. Jude Children's Research Hospital Memphis Tennessee USA
3. Dana‐Farber Cancer Institute and Harvard Medical School Boston Massachusetts USA
4. National Cancer Institute Washington District of Columbia USA
5. Duke University School of Medicine Durham North Carolina USA
6. Fred Hutchinson Cancer Research Center Seattle Washington USA
7. The University of Texas MD Anderson Cancer Center Houston Texas USA
8. University of Chicago Comer Children's Hospital Chicago Illinois USA
Abstract
AbstractBackgroundDespite survival improvements, there is a paucity of data on neurocognitive outcomes in neuroblastoma survivors. This study addresses this literature gap.MethodsNeurocognitive impairments in survivors were compared to sibling controls from the Childhood Cancer Survivor Study (CCSS) using the CCSS Neurocognitive Questionnaire. Impaired emotional regulation, organization, task efficiency, and memory defined as scores ≥90th percentile of sibling norms. Modified Poisson regression models evaluated associations with treatment exposures, era of diagnosis, and chronic conditions. Analyses were stratified by age at diagnosis (≤1 and >1 year) as proxy for lower versus higher risk disease.ResultsSurvivors (N = 837; median [range] age, 25 [17–58] years, age diagnosed, 1 [0–21] years) were compared to sibling controls (N = 728; age, 32 [16–43] years). Survivors had higher risk of impaired task efficiency (≤1 year relative risk [RR], 1.48; 95% confidence interval [CI], 1.08–2.03; >1 year RR, 1.58; 95% CI, 1.22–2.06) and emotional regulation (≤1 year RR, 1.51; 95% CI, 1.07–2.12; >1 year RR, 1.44; 95% CI, 1.06–1.95). Impaired task efficiency associated with platinum exposure (≤1 year RR, 1.74; 95% CI, 1.01–2.97), hearing loss (≤1 year RR, 1.95; 95% CI, 1.26–3.00; >1 year RR, 1.56; 95% CI, 1.09–2.24), cardiovascular (≤1 year RR, 1.83; 95% CI, 1.15–2.89; >1 year RR, 1.74; 95% CI, 1.12–2.69), neurologic (≤1 year RR, 2.00; 95% CI, 1.32–3.03; >1 year RR, 2.29; 95% CI, 1.64–3.21), and respiratory (>1 year RR, 2.35; 95% CI, 1.60–3.45) conditions. Survivors ≤1 year; female sex (RR, 1.54; 95% CI, 1.02–2.33), cardiovascular (RR, 1.71; 95% CI, 1.08–2.70) and respiratory (RR, 1.99; 95% CI, 1.14–3.49) conditions associated impaired emotional regulation. Survivors were less likely to be employed full‐time (p < .0001), graduate college (p = .035), and live independently (p < .0001).ConclusionsNeuroblastoma survivors report neurocognitive impairment impacting adult milestones. Identified health conditions and treatment exposures can be targeted to improve outcomes.Plain Language Summary
Survival rates continue to improve in patients with neuroblastoma.
There is a lack of information regarding neurocognitive outcomes in neuroblastoma survivors; most studies examined survivors of leukemia or brain tumors.
In this study, 837 adult survivors of childhood neuroblastoma were compared to siblings from the Childhood Cancer Survivorship Study.
Survivors had a 50% higher risk of impairment with attention/processing speed (task efficiency) and emotional reactivity/frustration tolerance (emotional regulation).
Survivors were less likely to reach adult milestones such as living independently.
Survivors with chronic health conditions are at a higher risk of impairment.
Early identification and aggressive management of chronic conditions may help mitigate the level of impairment.
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