Angiocentric glioma

Angiocentric glioma is a rare, low-grade brain tumor (WHO grade I) that was first recognized as a distinct entity by the World Health Organization in 2007. It is a slow-growing neuroepithelial tumor that predominantly affects children and young adults, typically arising in the cerebral cortex, most commonly in the temporal, frontal, or parietal lobes. The tumor is characterized by a distinctive histological pattern in which tumor cells are arranged around blood vessels (angiocentric growth pattern). Angiocentric glioma is also sometimes referred to as angiocentric neuroepithelial tumor (ANET) or monomorphous angiocentric glioma. The hallmark clinical presentation of angiocentric glioma is intractable epilepsy, often with a long history of drug-resistant seizures that may have begun in early childhood. Patients typically present with focal seizures that are refractory to antiepileptic medications. On MRI, the tumor usually appears as a cortical lesion with characteristic T1 hypointensity and T2/FLAIR hyperintensity, often with a stalk-like extension toward the ventricle. The primary treatment for angiocentric glioma is surgical resection. When gross total resection is achieved, the prognosis is generally excellent, with most patients experiencing seizure freedom and very low rates of tumor recurrence. Because of its low-grade, indolent nature, chemotherapy and radiation therapy are typically not required. In cases where complete resection is not feasible due to the tumor's location, subtotal resection may still provide significant improvement in seizure control. Long-term follow-up with neuroimaging is recommended to monitor for any recurrence. Overall, angiocentric glioma carries a favorable prognosis compared to many other brain tumors.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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