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Tectal Low-Grade Glioma with H3 K27M Mutation
Koichiro Sumi1, Katsunori Shijo1, Takahiro Igarashi1
1Division of Neurosurgery, Department of Neurological Surgery, Nihon University School of Medicine, Tokyo, Japan.
World Neurosurgery
|June 8, 2020
Summary
A rare pediatric pilocytic astrocytoma with H3 K27M mutation challenges current brain tumor classification. Further research is needed to understand the H3 K27M mutation's role in these gliomas.
Area of Science:
- Neuro-oncology
- Molecular Pathology
- Pediatric Oncology
Background:
- The 2016 WHO classification introduced "diffuse midline glioma, H3 K27M-mutant" as a new entity.
- This classification grades these gliomas as IV regardless of morphology, causing diagnostic confusion.
- The role of H3 K27M mutations in atypical gliomas remains unclear.
Observation:
- A rare case of pediatric low-grade glioma in the tectum is presented.
- The tumor exhibited pilocytic astrocytoma morphology but harbored the H3 K27M mutation.
- No microvascular proliferation, necrosis, mitoses, or other genetic alterations were observed.
Findings:
- The presented case, a pilocytic astrocytoma with H3 K27M mutation, could be classified as diffuse midline glioma, H3 K27M-mutant, Grade IV under current WHO guidelines.
- Despite its classification, the patient showed no neurological deficits 28 months post-treatment, with stable tumor size on MRI.
- This highlights a potential discrepancy between histological grade and clinical behavior in H3 K27M-mutant gliomas.
Implications:
- The findings suggest that circumscribed gliomas, such as pilocytic astrocytomas, can harbor the H3 K27M mutation.
- The clinical and biological significance of the H3 K27M mutation in pilocytic astrocytomas remains to be elucidated.
- Further studies are required to understand the biology and determine optimal treatments for these morphologically benign yet genetically altered gliomas.
