Current clinical practice about pediatric midline gliomas in the scope of molecular era

Abstract

AIM: To share our clinical experience with surgical and adjuvant treatment strategies followed during the treatment of midline gliomas. MATERIAL and METHODS: Pediatric patients with midline gliomas who underwent surgery in our clinic between March 2016 and November 2019 were included. Tissue samples were obtained through surgical excision, open biopsy, or stereotactic biopsy. All samples were analyzed for ATRX, BRAFV600E, IDH1/2, H3K27M, and H3G34R mutations, EGFR and PGFRA amplifications, and PTEN loss. RESULTS: There were 7 (43.8%) female and 9 (56.2%) male pediatric patients in the study. Eight patients had thalamic, 5 patients had pontine, 2 patients had medulla oblongata and one patient had brachium pontis tumors. Presenting symptoms were headache, disequilibrium, ophthalmoplegia, and panic attack. Eleven tumors showed H3K27M mutation and were diagnosed as diffuse midline gliomas. BRAFV600E, ATRX mutations, PTEN loss, and EGFR amplifications were other molecular alterations detected within tumor samples. Patients with H3K27M mutant tumors had a shorter life span. Five patients were enrolled in an ONC201 trial. CONCLUSION: Although most midline gliomas are not amenable to gross total excision, obtaining tissue samples is mandatory for determining patients' exact diagnoses, tailored treatment plans, and eligibility for clinical trials. Stereotactic biopsy for midline gliomas is a safe and effective method.

The Long Non-Coding RNA H19 Drives the Proliferation of Diffuse Intrinsic Pontine Glioma with H3K27 Mutation

“…The World Health Organization has recently classified DIPGs in the broader category of diffuse midline gliomas (DMGs) with a H3K27M mutation [2,[5][6][7]. Another frequent mutation of the H3 locus, GR4R, is more prevalent in pedHGGs located in the cerebral hemispheres [8]. The surgical removal of DIPGs is almost impossible due to their infiltrative nature and their location.…”

The long non-coding RNAH19drives the proliferation of diffuse intrinsic pontine glioma with H3K27 mutation

“…19.452516 doi: bioRxiv preprint recently classified DIPGs in the broader category of diffuse midline gliomas (DMG) with H3K27M mutation (2,(5)(6)(7). Another frequent mutation of the H3 locus, GR4R is more prevalent on pedHGGs located in the cerebral hemispheres (8). The surgical removal of DIPGs is almost impossible due to their infiltrative nature and to their location.…”