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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.
Ki-67 index is usually <5% 1.
MYB-QKI gene fusion 8
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.
hyperintense rim may be seen 4,9
extension toward the ventricles along vessels
may have cystic-appearing areas
T1 C+ (Gd): no enhancement
History and etymology
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:
"bubbly" cortical and subcortical lesion
rim of high signal on FLAIR
minimal surrounding edema
total nulling on the FLAIR sequence
subcortical cluster of cystic-like lesions
- 1. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central Nervous System. 4th Edition Revised" ISBN: 9789283244929
- 2. Wang M, Tihan T, Rojiani AM et-al. Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. J. Neuropathol. Exp. Neurol. 2005;64 (10): 875-81. Pubmed citation
- 3. Osborn AG, Salzman KL, Thurnher MM et-al. The new World Health Organization Classification of Central Nervous System Tumors: what can the neuroradiologist really say?. AJNR Am J Neuroradiol. 2012;33 (5): 795-802. AJNR Am J Neuroradiol (full text) - doi:10.3174/ajnr.A2583 - Pubmed citation
- 4. Shakur SF, McGirt MJ, Johnson MW et-al. Angiocentric glioma: a case series. J Neurosurg Pediatr. 2009;3 (3): 197-202. doi:10.3171/2008.11.PEDS0858 - Free text at pubmed - Pubmed citation
- 5. Lellouch-Tubiana A, Boddaert N, Bourgeois M et-al. Angiocentric neuroepithelial tumor (ANET): a new epilepsy-related clinicopathological entity with distinctive MRI. Brain Pathol. 2006;15 (4): 281-6. Pubmed citation
- 6. Frangoise Gray, Charles Duyckaerts, Umberto De Girolami. Escourolle and Poirier's Manual of Basic Neuropathology. ISBN: 9780199929054
- 7. Louis D, Perry A, Wesseling P et al. The 2021 WHO Classification of Tumors of the Central Nervous System: A Summary. Neuro-Oncology. 2021;23(8):1231-51. doi:10.1093/neuonc/noab106 - Pubmed
- 8. Bale T & Rosenblum M. The 2021 WHO Classification of Tumors of the Central Nervous System: An Update on Pediatric Low‐grade Gliomas and Glioneuronal Tumors. Brain Pathol. 2022;32(4):e13060. doi:10.1111/bpa.13060 - Pubmed
- 9. Cheng S, Lü Y, Xu S, Liu Q, Lee P. Cystoid Angiocentric Glioma: A Case Report and Literature Review. J Radiol Case Rep. 2015;9(7):1-9. doi:10.3941/jrcr.v9i7.2413 - Pubmed