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Angiocentric Glioma: A Case Series.
J Neurosurg Pediatr
  • Sophia F Shakur
  • Matthew J McGirt
  • M W Johnson, MD, Lehigh Valley Health Network
  • Peter C Burger
  • Edward Ahn
  • Benjamin S Carson
  • George I Jallo
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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.

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Citation Information

Shakur, S. F., McGirt, M. J., Johnson, M. W., Burger, P. C., Ahn, E., Carson, B. S., & Jallo, G. I. (2009). Angiocentric glioma: a case series. Journal Of Neurosurgery. Pediatrics, 3(3), 197-202. doi:10.3171/2008.11.PEDS0858