Angiocentric glioma: a case series.

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.