Angiocentric glioma

Definition

A rare diffuse glial neoplasm of the central nervous system characterized by a slowly progressing, grade 1 tumor usually arising in the cerebral cortex and/or brainstem, with tumor cells focally organized around the blood vessels in a distinctive pattern. Angiocentric glioma typically affects young patients and is clinically characterized by refractory seizures and headaches. Nearly all angiocentric gliomas exhibit MYB alterations (most commonly MYB::QKI fusion). Most cases are cured by surgical resection alone and the prognosis is usually favorable.