angiocentric glioma

angiocentric glioma

An indolent supratentorial glioma with perivascular distribution of neoplastic cells. 

Clinical findings
Patients usually present with drug-resistant focal epilepsy, usually of childhood onset.

Imaging
• FLAIR imaging—Solid, circumscribed, hyperintense, non-enhancing.
• MRI—T1-weighted images show cortical rim-like hyperintensities. Stalk-like extensions toward the ventricles in T2-weighted images and FLAIR are typical.
References in periodicals archive ?
Potential diagnoses included an infiltrating astrocytoma, an angiocentric glioma, and a diffuse variant of a low-grade astrocytoma such as a pilocytic astrocytoma.
MYB-QKI rearrangements in angiocentric glioma drive tumorigenicity through a tripartite mechanism.
37) Additionally, alterations in MYB and MYBL1, primarily fusion events, have been observed in angiocentric gliomas and low-grade astrocytomas.
Newly Codified Glial Neoplasms of the 2007 WHO Classification of Tumours of the Central Nervous System: Angiocentric Glioma, Pilomyxoid Astrocytoma and Pi tuicytoma.
These tumors were subsequently codified as a distinct clinicopathologic entity in the 2007 World Health Organization (WHO) Classification of Tumours of the Central Nervous System (3) and were given the name angiocentric glioma.
The pathology files were searched during a 20-year period of time (1989-2009) for tumors diagnosed as angiocentric glioma or tumors with pathologic descriptions potentially representing angiocentric glioma.
Angiocentric glioma is a low-grade (WHO grade I) cerebrocortical tumor of childhood with a characteristic angiocentric growth pattern.
Histopathologically, angiocentric glioma exhibits a perivascular growth pattern of bipolar spindle cells with mild pleomorphism, an infiltrative border, and lack of high-grade features such as elevated mitotic activity, necrosis, vascular proliferation, and meningeal extension.
The histogenesis of angiocentric glioma is a subject of debate, with postulated ependymal or radial glial cell origin.
The differential diagnosis of angiocentric glioma should include ependymoma and pilomyxoid astrocytoma.
Summary of Angiocentric Glioma Clinical Features Age At Case Diagnosis, No.
More than 90% of reported patients with angiocentric gliomas have presented with seizures.