Brainstem angiocentric glioma: report of 2 cases

Abstract

<jats:p>Angiocentric glioma is a rare tumor that was recognized by the <jats:italic>WHO Classification of Tumours of the Central Nervous System</jats:italic> as a distinct clinicopathological entity in 2007. Since this initial description, the vast majority of cases of angiocentric glioma reported in the literature have involved tumors of the cerebral hemispheres. To date, only 1 case of angiocentric glioma arising from the posterior midbrain has been reported. The authors present the cases of 2 pediatric patients who were found to have brainstem angiocentric gliomas. The clinical course, radiological and pathological features, treatment, and follow-up are described. The first case is one of a 5-year-old girl who presented with double vision, headache, and nausea and was found to have a midbrain lesion with pathological features consistent with angiocentric glioma. She was treated with resection and endoscopic third ventriculostomy (ETV), followed by close observation and serial neuroimaging. The second case is one of a 6-year-old boy who presented with progressive mouth drooping and problems with balance. He was found to have a pontine lesion with pathological features consistent with angiocentric glioma. This patient was treated with ETV, followed by close observation and serial neuroimaging. This report includes 6 and 1.5 years of follow-up of the patients, respectively. While there are limited data regarding the prognosis or long-term management of patients with brainstem angiocentric gliomas, the cases described in this report suggest an indolent course for this tumor, similar to the course of angiocentric gliomas located in the cerebral hemispheres.</jats:p>

Clinical characteristics, treatment and prognosis of angiocentric glioma

“…Only 15 patients exhibited different symptoms. Of these 15 patients, eight had headaches, four of which had decreased vision ( 7 , 12 , 47 ), three experienced dizziness, one of which had otalgia ( 9 , 19 , 49 ), two had ataxia ( 11 , 47 ), two had swallowing disorders ( 34 , 37 ), two presented with weakness and numbness of the left side of the body ( 17 , 45 ) and one had strabismus ( 34 ). The majority of the AG tumors (94.4%; 102/108) were in a supratentorial location situated within/under the cerebral cortex and 81.5% (88/108) were located in a single lobe.…”

“…The majority of the AG tumors (94.4%; 102/108) were in a supratentorial location situated within/under the cerebral cortex and 81.5% (88/108) were located in a single lobe. A total of six tumors were located in the brainstem ( 11 , 34 , 37 , 47 ). In a total of 46 cases, the tumor was in the left brain and in 43 cases, it was in the right brain.…”

“…A total of 64.9% (61/94) of the patients with AG underwent GTR and 27.7% (26/94) underwent subtotal resection (STR). Furthermore, 7 cases only had a biopsy ( 7 , 34 , 37 , 47 ). The follow-up time ranged between 0.25 and 168 months (mean, 23.5 months).…”

Angiocentric Glioma: A Review of Clinicopathologic Features

“…The most common sites in early reported series were fronto-parietal lobes followed by the temporal lobe [4][5][6]. Tumors have been described involving the occipital lobe, as well as rarely the brainstem [9][10][11].…”

“…Occasionally, based on the location of the tumor, patients may present with other symptoms. Reports of progressive hand weakness [8], headaches and visual disturbance [9,12], double vision and nausea [11], hemiparetic gait and facial nerve palsy [11], and psychotic symptoms [13] have been documented in the literature.…”