Epilepsy characteristics and outcomes in patients with pleomorphic xanthoastrocytomas

BACKGROUND: Pleomorphic xanthoastrocytomas (PXAs) are rare brain tumors frequently associated with tumor-related epilepsy. Few studies have analyzed epilepsy outcomes in relation to tumor characteristics, clinical features, or neurophysiologic findings in this patient population. METHODS: This is a retrospective study of 25 patients diagnosed with a PXA at a single institution between 2004 and 2025. Clinical, pathologic, demographic, and treatment data were reviewed to identify factors associated with seizure outcomes after surgical resection. RESULTS: Most patients (19/25, 76%) had tumor-related epilepsy with 13/19 (68.4%) presenting with seizure as the initial symptom. In retrospect, 6/19 (31.6%) patients had unrecognized focal seizures prior to the index presentation. After the first tumor resection prior to tumor recurrence, 15/19 (78.9%) patients were seizure free. At the most recent follow-up visit, 13/19 (68.4%) patients were seizure free. Seizure freedom at the most recent follow up was observed in 7/8 (87.5%) patients with ATRX loss compared with 1/5 (20%) without ATRX loss (p = 0.01). Of the patients with seizure recurrence, 5/6 (83.3%) had recurrence of tumor on MRI within 30 days of the breakthrough seizure (p = 0.003). CONCLUSIONS: Most patients with PXA-associated tumor-related epilepsy achieved seizure freedom after initial resection. Seizure recurrence was strongly associated with tumor recurrence at long-term follow up. ATRX loss was associated with seizure freedom at the most recent follow up, suggesting that tumor molecular features may help prognosticate epilepsy outcomes. Nearly one-third of patients had previously unrecognized focal seizures highlighting the importance of a detailed, anatomically guided seizure history.