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Published on: January 14, 2014
Angiocentric glioma: a case series
Sophia F Shakur1, Matthew J McGirt, Michael W Johnson
1Departments of Neurosurgery John Hopkins University School of Medicine, Baltimore, Maryland, USA.
Insights
Pediatric angiocentric glioma, a rare brain tumor, often presents with seizures. Complete surgical removal of these temporal lobe tumors leads to excellent outcomes and seizure freedom in young patients.
Area of Science:
- Neuro-oncology
- Pediatric Neurology
- Brain Tumor Classification
Background:
- Angiocentric glioma is a distinct clinicopathological entity recognized in the 2007 WHO Classification.
- This study presents the first 3 pediatric cases from the authors' institution.
Purpose of the Study:
- To describe the characteristics and outcomes of pediatric angiocentric glioma.
- To review the literature for reported pediatric cases.
Main Methods:
- Case series of 3 pediatric patients (ages 10-13) with angiocentric glioma.
- Literature review of 22 additional pediatric cases, totaling 25 cases.
- Analysis of clinical presentation, neuroimaging (MRI), histology, and treatment outcomes.
Main Results:
- Common presentation: intractable seizures. One case presented with headache and vision loss.
- MRI findings: superficial, nonenhancing, T2-hyperintense temporal lobe lesions.
- Histology: monomorphic cells with perivascular orientation, glial markers positive.
- All 3 patients achieved gross-total resection and remained seizure-free post-surgery.
Conclusions:
- Seizures are the most frequent symptom of pediatric angiocentric glioma.
- Characteristic MRI findings include supratentorial, nonenhancing lesions.
- Gross-total resection is associated with excellent patient outcomes.
Object:
Angiocentric glioma was recently recognized as a distinct clinicopathological entity in the 2007 World Health Organization Classification of Tumours of the Central Nervous System. The authors present the first 3 pediatric cases of angiocentric glioma encountered at their institution and review the literature of reported cases to elucidate the characteristics and outcomes of pediatric patients with this novel tumor.
Methods:
The children in the 3 cases of angiocentric glioma were 10, 10, and 13 years old. Two presented with intractable seizures and 1 with worsening headache and several months of decreasing visual acuity. Twenty-five cases, including the 3 first described in the present paper, were culled from the literature.
Results:
In all 3 cases, MR imaging demonstrated a superficial, nonenhancing, T2-hyperintense lesion in the left temporal lobe. Histologically, the tumors were composed of monomorphous cells with a strikingly perivascular orientation that were variably reactive for glial fibrillary acidic protein and epithelial membrane antigen. Surgical treatment resulted in gross-total resection in all 3 cases. By 24, 9, and 6 months after surgery, all 3 patients remained seizure free without focal neurological deficits.
Conclusions:
Among 25 cases of angiocentric glioma, seizure was the most common symptom at presentation. Magnetic resonance imaging demonstrated supratentorial, nonenhancing, T1-hypointense, T2-hyperintense lesions. Gross-total resection of this lesion yields excellent results.
