Primary Mismatch Repair Deficient Glioma (PMMRDG), IDH-wildtype and H3-wildtype: A Giant Cell Tumor with Potential for Long-Term Survival Occurring at all Ages - PubMed
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- #PMMRDG
- #immune activation
- #long-term survival
- Primary Mismatch Repair Deficient Gliomas (PMMRDGs) are IDH-wildtype and H3-wildtype gliomas linked to inherited DNA repair issues, occurring in all age groups.
- These tumors are characterized by a high frequency of replication repair deficiency, multinucleated giant cells, and loss of MMR protein expression.
- Patients with high mutational burden (>50 mut/Mb) and intact chromosome 9p region show long-term survival, likely due to immune activation.
- PMMRDGs form distinct DNA methylation clusters and represent a unique glioma type with potential for improved treatment strategies.