Angiocentric glioma

Last revised by Arlene Campos on 12 Jan 2024

Angiocentric gliomas are rare, superficial slow-growing WHO grade 1 brain tumors typically presenting in pediatric patients with intractable focal epilepsy 1-6

Angiocentric gliomas are very rare tumors with relatively few reported cases. They usually affect children and young adults 1,6. No reported gender predilection has been reported 1

Cortical dysplasia may be associated.

Seizures are the classical presentation, with over 95% of patients presenting with intractable seizures 3.

Angiocentric gliomas are considered pediatric-type diffuse low-grade gliomas and designated as WHO grade 1 tumors in the current WHO brain tumor classification 1-3,7. The exact etiology of angiocentric gliomas remains unclear although some features are similar to ependymomas 1,6. In fact, sometimes, a distinct ependymoma component may co-exist 1.

These tumors demonstrate a monomorphic population of elongated spindle-shaped bipolar cells with a strikingly perivascular orientation, somewhat reminiscent of perivascular pseudorosettes 1,6. Although tumor cells do extend into the surrounding parenchyma, a strong predilection for perivascular spread is evident 6. Subpial growth along the surface of the cortex is also a prominent feature 1,6.

The immunophenotype shares some similarities to ependymomas 1

  • GFAP: positive

  • EMA: both surface and perinuclear cytoplasmic dot may be present

Ki-67 index is usually <5% 1

They do not demonstrate neuronal markers (e.g. synaptophysin, chromogranin-A, neuronal nuclear antigen) and they are isocitrate dehydrogenase (IDH) negative.

  • MYB-QKI gene fusion 8

  • IDH-wildtype

Angiocentric gliomas are usually cortical or subcortical (grey-white matter junction), typically well-delineated, supratentorial tumors that tend to expand affected gyri. They exhibit a propensity to spread horizontally in the subpial plane and deeply along vessels 1.

Typically appear as an expansile non-enhancing cortical tumor.

  • T1

    • hypointense

    • hyperintense rim may be seen 4,9

  • T2/FLAIR

    • hyperintense

    • extension toward the ventricles along vessels

    • may have cystic-appearing areas

  • T1 C+ (Gd): no enhancement

Angiocentric glioma was initially identified in 2005 in two separate case reports 2,5 and then introduced in the WHO classification of CNS tumors in 2007.

Given their rarity, on purely imaging grounds it is difficult to distinguish these tumors from more common diffuse gliomas (both astrocytoma and oligodendroglioma). Other imaging differential considerations include:

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Cases and figures

  • Case 1
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