Cognitive outcomes among survivors of focal low-grade brainstem tumors diagnosed in childhood.

Pediatric focal low-grade brainstem tumors are associated with excellent prognosis. Surgical resection and conformal radiation therapy are front-line treatment options; radiation therapy (RT) serves as an excellent treatment for disease progression. Given high survival rates and limited research regarding functional outcomes, the current study examined neurocognitive outcomes in a group of low-grade brainstem glioma survivors. Forty-three survivors of focal low-grade brainstem gliomas underwent neurocognitive assessment (58 % male; median = 6.9 years at diagnosis; median = 14.9 years at latest assessment). Treatment included combinations of surgery, chemotherapy, and RT with 70 % ultimately receiving RT. Neurocognitive outcomes were evaluated through retrospective chart review. Intellectual and academic performance were significantly different from normative expectations (full scale IQ = 86.5 ± 16.8; reading comprehension = 91.3 ± 16.4; math reasoning = 88.2 ± 18.9; reference group = 100 ± 15). Further, the percentage performing below average exceeded the expected 16 % in the normative sample (full scale IQ = 43 %; reading comprehension = 37 %; math reasoning = 50 %). Mean parent ratings did not reflect concerns regarding internalizing and externalizing behaviors or executive functioning (internalizing = 54.9 ± 12.7; externalizing = 51.6 ± 14.6, global executive composite = 57.1 ± 16.0; reference group = 50 ± 10); however, the proportion with clinically elevated scores was higher than the expected 16 % (internalizing = 42 %; externalizing = 26 %; global executive composite = 38 %). Mean performance fell below average for visual-motor coordination (81.8 ± 13.2) and parent ratings of adaptive functioning (73.4 ± 24.2), with 65 and 62 % falling outside the average range, respectively. There were no significant differences between those receiving and not receiving RT. Multiple cognitive domains were significantly different from normative expectations. Despite focal disease and treatment targeting subcortical brain regions, neurocognitive risks exist that may impact treatment planning and caregiver education.