Angiocentric Glioma-Like Tumor of the Midbrain

Abstract

Angiocentric glioma, a rare brain neoplasm with features of ependymal differentiation, has only recently been recognized as a distinct clinicopathological entity. To date, all reported cases have involved tumors in the cerebral hemispheres, and the majority have presented with seizures. The authors report the case of a 5-year-old girl who presented with several cranial neuropathies and mild gait disturbance. An exophytic neoplasm arising from the posterior midbrain and causing obstructive hydrocephalus was identified, and surgical resection revealed a neoplasm with features of angiocentric glioma.

Angiocentric glioma from a perspective of A-B-C classification of epilepsy associated tumors

“…Despite that, supposedly malignant variants of AG (possibly WHO III), characterized by a higher mitotic index, vascular proliferation and necroses were also observed [1,15]. The elective method of treatment is gross total resection, but subtotal resection and chemotherapy and radiotherapy had also been used, especially in more difficult locations or to handle rare, high grade variants [1,5,16,20]. One has to acknowledge that the neuroradiological picture, though characteristic, is not definitely specific therefore at least biopsy is mandatory.…”

Angiocentric Glioma and Surrounding Cortical Dysplasia Manifesting as Intractable Frontal Lobe Epilepsy-Case Report-

“…A single case of midbrain tumor with histological features similar to angiocentric glioma was also reported. 2) No recurrence or progression has been reported after gross total or subtotal removal of tumor, except for one patient who died of tumor progression 62 months after the first surgery. 11) Ki-67 labeling index was 1% or less in 18 of 29 reported cases (62.1%).…”

“…[1][2][3][4][5][6][8][9][10][11] The defining histological features of this tumor are the striking perivascular pattern of growth, schwannoma-like palisade arrangement of tumor cells, and characteristic``dot-like'' EMA staining of microlumens of the tumor, suggesting ependymal differentiation in part. The neuroimaging features include solid T 2 -weighted hyperintense lesion in the superficial cerebrum with no contrast enhancement.…”

“…Since then, a total of 36 cases have been reported. [1][2][3][4][5][6][8][9][10][11] Angiocentric glioma is a slow-growing supratentorial tumor with characteristic radiological features, frequently manifesting as recurrent seizures in children and young adults. Histologically, the tumor is characterized by a perivascular pattern of growth and dotlike staining of microlumens of tumor cells with epithelial membrane antigen (EMA).…”

Original article Angiocentric glioma: a rare intractable epilepsy-related tumour in children

“…Majority of them are located superficially in the frontal, temporal or parietal lobes, as well as in the hippocampal region. The case with features of angiocentric glioma in the posterior midbrain was also described [7]. Although AGs are typically localized within the cerebral cortex, they may extend into the adjacent white matter.…”