QOL-24. NEUROLOGIC AND NEUROCOGNITIVE OUTCOMES AMONG SURVIVORS OF INFANT-TYPE HEMISPHERIC GLIOMA (IHG)

Abstract IHGs are large, hemorrhagic cerebral tumors associated with hydrocephalus, increased intracranial pressure, and herniation. Commonly diagnosed during early infancy, children with IHGs have an approximately 90% chance of overall survival when treated with surgery, chemotherapy, and, more recently, molecularly targeted therapies. However, there is limited understanding of long-term quality-of-life following treatment. Our study investigated long-term neurological and neurocognitive outcomes among IHG survivors. The medical database was reviewed for neurological sequelae and neurocognitive outcomes (intelligence [IQ], adaptive function, academic readiness) for 21 survivors. Median age of diagnosis and last follow-up were 0.8 (0.0-4.4) and 10.3 (4.8-17.6) years, respectively. Sixteen patients (76%) experienced atleast one neurological sequelae: 38% (8/21) had paresis/plegia; 24% (5/21) had spasticity, 26% (5/19) had abnormal speech, and 57% (12/21) had a long-term seizure disorder. Nine patients diagnosed with IHG at < 0.5 years had significantly higher rates of dysarthria (p = 0.019) and seizure disorders (p = 0.035) in follow-up in comparison to patients diagnosed at 0.5-1 year (n=4) and >1 year (n=8). Neurocognitive outcomes were available for 19 patients at a median of 6 years after diagnosis. Median IQ for the entire cohort was 68.0 (47.0-114.0), compared to normative expectations of 100 (SD = 15), with younger age at diagnosis associated with lower IQ (r = 0.36). Ten survivors experiencing seizures particularly had worse IQ scores (median 69.4 [47-101]). Survivors also had inferior outcomes in school readiness (median 46.0 [40.0-98.0]), compared to normative expectations of 100 (SD = 15), and adaptive function (median 48.0 [34.0-67.0]), compared to normative expectations of 50 (SD= 10), with younger age at diagnosis associated with lower performance for adaptive function (r = 0.45). CONCLUSION Children diagnosed with IHG have a high survival rate but experience substantial tumor-associated and treatment-related long-term morbidity. Future clinical trials should aim for better long-term quality of life for survivors through scientifically driven treatment modification.