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

Abstract

Objective 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. Methods 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. Results 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. Conclusions 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.

Neuropsychological outcomes of patients with low-grade glioma diagnosed during the first year of life

“…Additionally, infants with a greater number of tumor directed surgeries displayed poorer dominant hand fine motor functioning. Children with LGGs undergoing surgery alone display visual-motor deficits [9, 46], and previous studies have demonstrated that the number and extent of surgical resections are associated with neurocognitive performance [47, 48]. Subsequent surgeries likely infer increased risk of complications and brain injury.…”

“…However, these findings are largely based on studies of children treated with craniospinal or whole brain irradiation [14]. Cognitive late effects following focal radiation are less conclusive, and several studies of children treated for LGGs have found limited impact of radiation therapy on neurocognitive outcomes [12, 46]. It is likely that extent of cognitive late effects resulting from radiation therapy is limited in many of these cases due to the neurocognitive hits these children have previously received resulting from multiple surgeries and chemotherapy regimens.…”

“…To thoroughly evaluate these deficits, future studies would benefit from sensitive measures of speeded processing, attention, and working memory. Additionally, assessment of more distal functional outcomes such as quality of life and achievement of milestones including college graduation, employment, and independent living would help to further understand the functional outcomes for this group [46]. …”

Adaptive behavior impaired in children with low‐grade glioma

“…Given the high long‐term survival rates and adverse long‐term health outcomes, skills to cope with daily life challenges need attention during follow‐up, especially as children with LGG are at risk for impaired adaptive behavior, i.e., the successful performance of age‐appropriate daily activities required for personal and social independence . Children with LGG are also at risk for a longitudinal decline in communication skills .…”

Adaptive functioning in pediatric brain tumor survivors: An examination of ethnicity and socioeconomic status

“…9,20,23 Children treated for infratentorial and supratentorial tumors have been shown to demonstrate worse adaptive functioning compared with sibling and solid tumor control groups 9 and to the normative mean, 10 as well as increased rates of impairment. 20 Broadly weaker adaptive functioning 24,25 as well as weaknesses in specific adaptive skill areas including communication, 10 socialization( 10 but see 20 ), and practical (e.g., self-care, health/safety skills, home living skills) 20 have been documented.…”