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Abstract Details

Incidence and Natural History of H3 K27M-mutated Midline Gliomas in Adults
Neuro-oncology
S30 - Brain Cancer: From Epidemiology to Quality of Life (3:30 PM-3:41 PM)
001

H3F3A mutations define the entity of Diffuse Midline Glioma, which was added to the WHO 2016 classification. There have been several reports describing the clinical, prognostic, and histopathological implications of this mutation in children. It is unclear, however, what proportion of adults with gliomas occurring in the midline have an H3 K27M mutation. We set out to define this in a single-institution, retrospective cohort study.

Define incidence and prognosis of H3 K27M mutated gliomas in adults as compared to non-mutated midline gliomas.

From 850 consecutive gliomas in adults we identified 163 cases with midline gliomas on MRI (parasaggital cortex, corpus callosum, basal ganglia, thalamus, brainstem, spinal cord, or cerebellum). Clinical cases were reviewed in accordance with IRB guidelines. FFPE tissue was obtained from 123 cases and stained for H3 K27M, IDH1, and ATRX.

A H3 K27M mutation was identified in 18 of 123 cases (15%). As compared to non-H3 K27M mutated tumors, there were no difference in age at diagnosis, sex, tumor grade at diagnosis, contrast enhancement on MRI, extent of resection, or treatment received. All H3 K27M mutant tumors were WHO grade III or IV on histology, while non-mutant tumors encompassed all four grades (p = 0.13). The most common locations to have H3 K27M-mutated tumors were midbrain (2/3; 67%), pons (4/11; 36%), cerebellum (6/24; 25%), spinal cord (2/14; 14%), and thalamus (3/31; 10%). Median survival was significantly longer for patients with H3 K27M-mutated tumors (17.6 months) than high grade non-mutated tumors (7.7 months, p = 0.03).

H3 K27M mutations are common in midline gliomas in adults, thus this molecular subtype should be considered in adults of all ages and tumor grades. H3 K27M mutations are associated with improved survival in adults, which is quite different than in pediatric patients where H3 K27M mutations confer a worse prognosis. 
Authors/Disclosures
Karisa C. Schreck, MD, PhD
PRESENTER
Dr. Schreck has received personal compensation in the range of $0-$499 for serving as a Consultant for Nurix. Dr. Schreck has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Schreck has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Advarra LLC. The institution of Dr. Schreck has received research support from Springworks Therapeutics. Dr. Schreck has received personal compensation in the range of $500-$4,999 for serving as a speaker with Springworks Therapeutics.
Surabhi Ranjan, MD Dr. Ranjan has nothing to disclose.
No disclosure on file
Matthias Holdhoff No disclosure on file
No disclosure on file