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Spinal Cord Diffuse Midline Gliomas With H3 K27m-Mutant: Clinicopathological Features and Prognosis
Yong-Zhi Wang1, Yao-Wu Zhang1, Wei-Hao Liu1
1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, No. 119 South 4th Ring West Road, Fengtai District, People's Republic of China.
Background:
"Diffuse midline glioma, H3 K27M-mutant" (DMG) mainly arises within the pontine, thalamic, and spinal cord regions. Because of the rarity of spinal cord gliomas, the general knowledge surrounding DMGs is mainly based on pontine and thalamic gliomas, whereas tumor location tends to influence the clinicopathological features and prognosis.
Objective:
To determine the clinicopathological characteristics and molecular profiles of DMGs located in the spinal cord.
Methods:
The clinical and molecular pathologic features and prognosis were comprehensively analyzed in a series of 44 patients with spinal cord DMGs.
Results:
The median age was 36 yr, and 88.7% of patients (39/44) were adults (≥18 yr). Histopathologically, malignant grades included grade II (16 cases), grade III (20 cases), and grade IV (8 cases). Compared with patients with histological grade IV, patients with lower histological grade (grade II/III) were older (37 vs 24 yr, P = .020) and were associated with longer overall survival (24.1 vs 8.6 mo, P = .007). All 30 tested tumors were isocitrate dehydrogenase (IDH) wild type, and 96% of cases (22/23) presented with unmethylated O6-methylguanine-DNA methyltransferase. Univariate and multivariate analyses showed that histological grade and presurgery McCormick Scale scores were independent prognostic factors for overall survival, whereas extensive surgical resection and chemoradiotherapy were not significantly associated with improved survival. The most frequent anatomic locations were the cervical enlargement (C4-T1, n = 16) and conus medullaris (T12-L1, n = 13), which exhibited distinctive clinical characteristics and molecular features.
Conclusion:
The findings provide guidelines for the evidence-based practice of the specialized management of spinal cord DMGs.
Insights
Diffuse midline gliomas (DMGs) in the spinal cord are rare. Lower histological grades correlate with older age and longer survival, highlighting key prognostic factors for this rare tumor.
Area of Science:
- Neuro-oncology
- Molecular Pathology
- Clinical Neuroscience
Background:
- Diffuse midline gliomas (DMGs) predominantly occur in the brainstem and thalamus.
- Spinal cord DMGs are rare, and their characteristics are less understood compared to brainstem/thalamic counterparts.
- Tumor location significantly impacts clinicopathological features and prognosis in gliomas.
Purpose of the Study:
- To elucidate the clinicopathological characteristics of spinal cord DMGs.
- To define the molecular profiles of DMGs originating in the spinal cord.
- To identify prognostic factors specific to spinal cord DMGs.
Main Methods:
- Comprehensive analysis of clinical data from 44 patients with spinal cord DMGs.
- Evaluation of histopathological features, including tumor grade.
- Assessment of molecular markers (IDH status, MGMT methylation) and prognostic factors.
Main Results:
- The median age of patients was 36 years, with most being adults.
- Lower histological grades (II/III) were associated with older age and significantly longer overall survival compared to grade IV.
- Histological grade and pre-surgery McCormick Scale scores were independent prognostic factors; extensive surgery and chemoradiotherapy did not significantly improve survival.
Conclusions:
- Histological grade and pre-surgery McCormick Scale scores are critical prognostic indicators for spinal cord DMGs.
- Findings offer evidence-based guidance for the specialized management of spinal cord DMGs.
- Distinct clinical and molecular features were observed based on tumor location within the spinal cord.
