QOL-22. Neurocognitive performance in survivors of pediatric brain tumors with neurocutaneous syndromes

Abstract BACKGROUND: Pediatric brain tumors (PBT) in children with neurocutaneous syndromes are typically low-grade and survival rates are close to 100%. Neurocutaneous syndromes are associated with neurocognitive and behavioral problems, and a diagnosis of brain tumor adds to the total neurocognitive burden. The aim of this study was to describe neurocognitive performance in survivors of PBT with a neurocutaneous syndrome and to find associated factors. METHOD: Neurocognitive data (verbal and non-verbal reasoning, cognitive processing speed, working memory, and executive function) on 20 survivors of brain tumors with neurocutaneous syndromes were collected from medical logs. Results were converted to z-scores (m=0, sd=1) using age-adjusted national norms. Generalized linear models were built to find factors associated with neurocognitive performance. Most survivors had received several assessments and all assessments were added to the model together with time since diagnosis at assessment. RESULTS: Seventeen of the survivors (85%) had undergone 1-3 assessments and 3 survivors had undergone 4-5 assessments. Six survivors (30%) had a diagnosis of intellectual disability, and neurocognitive scores were low at all assessment points: average z-score across all datapoints(sd): verbal reasoning -1.1(1.2); non-verbal reasoning -1.2(0.9); working memory -1.9(0.7); cognitive processing speed -1.0 (1.0); executive function -0.6 (1.0)). Factors associated with worse neurocognitive performance were young age at brain tumor diagnosis, larger tumors, epilepsy, and longer time span between diagnosis and assessment. CONCLUSIONS: Survivors of PBT with neurocutaneous syndromes are at risk for neurocognitive deficits that might worsen over time. These survivors need personalized neurocognitive follow-up. When examining neurocognitive performance and associated factors in survivors of PBT special consideration needs to be taken to survivors with neurocutaneous syndrome since these survivors receive limited cancer treatment and still have neurocognitive deficits.