The prognosis of diffuse midline glioma H3 K27M mutant.

Abstract

e13548 Background: Diffuse Midline Glioma is rare spinal cord primary tumor. Unfortunately it is known that the prognosis is poor, and it was added to the WHO guidelines in 2016 as a grade IV. It is so rare that the prognosis of the diffuse midline glioma in spinal cord is not well known yet. So we want to analyze the prognosis of the spinal cord diffuse midline glioma and find out about the molecular profile. Methods: We retrospectively reviewed of 10 diffuse midline glioma H3-K27M mutant patients who underwent surgery in a single institute, 2015.09~2018.04. We analyzed the demographic data, overall survival, progression free survival and molecular profile which includes PTEN, BRAF, EGFR, MGMT methylation status, c-MET, IDH1, 1p/19q deletion. Results: Mean age was 44.8 yr; male to female ratio was 5:5. Tumor was located conus medullaris (8), CT Junction(1), Cervical(1). One was given gross total resection and the other was a partial resection or biopsy. All patient were treated with CCRT. Medial overall survival was 47.8 months, and median progression free survival was 21 months. One case each was found for BRAF, C-MET, IDH1, PTEN and EGFR. Conclusions: This study was conducted on patients collected for a relatively short period. This study reported survival analysis and molecular profile. These mutations are known to be mainly associated with MAPK pathway. So there may be a correlation between H3-K27M and MAPK pathway. Results indicating the association of MAPK and H3K27M can be found in some literature. Further research is needed.