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Current Clinical Practice About Pediatric Midline Gliomas in the Scope of Molecular Era
Bahattin Tanrikulu1, M Memet Ozek
1Acibadem Mehmet Ali Aydinlar University School of Medicine, Department of Neurosurgery, Istanbul, Turkey.
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.
Insights
Surgical and adjuvant treatments were evaluated for pediatric midline gliomas. H3K27M mutations were common and associated with shorter survival, highlighting the importance of molecular diagnostics for tailored treatment plans.
Area of Science:
- Pediatric neuro-oncology
- Cancer genomics
- Neurosurgery
Background:
- Midline gliomas are aggressive brain tumors in children.
- Treatment strategies often involve surgery and adjuvant therapies.
- Accurate molecular profiling is crucial for diagnosis and management.
Purpose of the Study:
- To report clinical experience with surgical and adjuvant treatments for pediatric midline gliomas.
- To analyze molecular alterations in tumor samples.
- To assess the impact of molecular findings on patient outcomes.
Main Methods:
- Retrospective analysis of pediatric patients with midline gliomas treated between March 2016 and November 2019.
- Surgical tissue acquisition via excision or biopsy.
- Comprehensive molecular analysis including ATRX, BRAFV600E, IDH1/2, H3K27M, H3G34R mutations, EGFR/PGFRA amplifications, and PTEN loss.
Main Results:
- Eleven of sixteen tumors exhibited H3K27M mutation, classifying them as diffuse midline gliomas.
- Other detected molecular alterations included BRAFV600E, ATRX mutations, PTEN loss, and EGFR amplifications.
- Patients with H3K27M mutant tumors demonstrated a reduced lifespan. Five patients participated in an ONC201 trial.
Conclusions:
- Gross total excision is often not feasible for midline gliomas.
- Tissue sampling is essential for precise diagnosis, personalized treatment, and clinical trial eligibility.
- Stereotactic biopsy is a safe and effective method for obtaining diagnostic tissue.
