Journal
NEUROSURGERY
Volume 89, Issue 2, Pages 300-307Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1093/neuros/nyab174
Keywords
Astrocytoma; Spinal cord; Intramedullary tumor; Diffuse midline glioma; H3 K27M mutation
Categories
Funding
- National Key Research and Development Program of China [2018YFC0115604]
- Strategic Priority Research Program of the Chinese Academy of Sciences [XDA16040303]
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The study analyzed 44 patients with spinal cord DMG and found that patients with lower histological grade were older and had longer overall survival. All 30 tested tumors were IDH wild type, and 96% of cases presented with unmethylated O-6-methylguanine-DNA methyltransferase.
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 O-6-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.
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