Clinical characteristics, treatment and prognosis of angiocentric glioma

Abstract

Angiocentric glioma (AG) is a rare subtype of neuroepithelial tumor in children and young adults that commonly presents with seizures. To study the clinical characteristics, treatment and prognosis of patients with AG, the features of two cases of AG were described and 108 cases reported in the literature were assessed. The cases of the present study were two males aged 8 and 16 years, who mainly presented with seizures. MRI revealed superficial, non-enhanced lesions in the left temporal and right frontal lobe, respectively. The two patients underwent gross total resection (GTR) and remained seizure-free without neurological deficits after 3.5 and 2.5 years, respectively. Histopathological examination revealed that the tumors consisted of monomorphous cells that surrounded the blood vessels and neurons in the cerebral cortex, and formed concentric sleeves or pseudorosettes. Furthermore, immunostaining indicated that the diffuse infiltrative neoplastic cells were positive for glial fibrillary acidic protein and a dot-like pattern of epithelial membrane antigen was observed. AG mostly appeared similar to low-grade gliomas on MRI. GTR of the lesions was curative and radiation or chemotherapy were not required. AG typically has a favorable prognosis, with low mortality and incidence of disability.

Imaging of brain tumors in children: the basics—a narrative review

“…Previous studies noted that a myeloblastosis quaking (MYB-QKI) gene rearrangement was a distinct feature observed in the majority of angiocentric gliomas, while IDH1 mutation was not noted (36,37). This molecular profile may help the differential diagnosis of angiocentric gliomas from other gliomas and IDH1-mutated glioblastomas (35).…”

“…They may be associated with cortical dysplasia, as well as DNETs (34). Angiocentric gliomas are most commonly located in the supratentorial compartment, particularly in the frontoparietal and temporal lobes as well as the hippocampal region (35).…”

Pediatric angiocentric glioma with acute intracerebral hemorrhage: A case report with 36 months follow-up

“…Two years later, the WHO officially confirmed it as a central nervous system entity tumor for the 1 st time in the classification of tumors of the central nervous system . [ 9 ] Considering the low incidence of this tumor, 108 cases had been reported until December 2019,[ 5 ] excluding this case we described. They had similar radiological features, such as magnetic resonance image (MRI) T1 hypointense signal, hyperintense signal in T2, and fluid-attenuated inversion recovery (FLAIR’s) without contrast enhancement.…”

“…They had similar radiological features, such as magnetic resonance image (MRI) T1 hypointense signal, hyperintense signal in T2, and fluid-attenuated inversion recovery (FLAIR’s) without contrast enhancement. [ 1 , 5 ]…”

“…[ 10 ] Drug-refractory epilepsy is the most common complaint in this tumor, other symptoms such as headache, vision deficit, dizziness, earache, ataxia, dysphagia, weakness, numbness, and strabismus had been reported. [ 5 ] Here, we reported a case of AG with acute intracerebral hemorrhage (ICH) that never has been described in the literature.…”