Current Clinical Practice About Pediatric Midline Gliomas in the Scope of Molecular Era

Özet

AIM: To share our clinical experience with surgical and adjuvant treatment strategies followed during the treatment of midlinegliomas.MATERIAL and METHODS: Pediatric patients with midline gliomas who underwent surgery in our clinic between March 2016 andNovember 2019 were included. Tissue samples were obtained through surgical excision, open biopsy, or stereotactic biopsy. Allsamples were analyzed for ATRX, BRAFV600E, IDH1/2, H3K27M, and H3G34R mutations, EGFR and PGFRA amplifications, andPTEN loss.RESULTS: There were 7 (43.8%) female and 9 (56.2%) male pediatric patients in the study. Eight patients had thalamic, 5 patientshad 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 midlinegliomas. BRAFV600E, ATRX mutations, PTEN loss, and EGFR amplifications were other molecular alterations detected within tumorsamples. 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 mandatoryfor determining patients’ exact diagnoses, tailored treatment plans, and eligibility for clinical trials. Stereotactic biopsy for midlinegliomas is a safe and effective method.