Seizures in the context of brain tumors are a relatively common symptom, with higher occurrence rates observed in glioneuronal tumors and gliomas. It is a serious burden that can have a significant impact on the quality of life (QoL) of patients and influence the disease's prognosis. Brain tumor‑related epilepsy (BTRE) is a challenging entity because the pathophysiological mechanisms are not fully understood yet. Nonetheless, neuroinflammation is considered to play a pivotal role. Next to neuroinflammation, findings on the pathogenesis of BTRE have established that certain genetic mutations are involved, of which the most known would be IDH mutations in gliomas. Others discussed more thoroughly in the present review include genes such as PTEN, TP53, IGSF3, and these findings all provide fresh and fascinating insights into the pathogenesis of BTRE. Treatment for BTRE presents unique challenges, mainly related to burdens of polytherapy, debated necessity of anti‑epileptic prophylaxis, and overall impact on the QoL. In fact, there are no established anti‑seizure medications (ASMs) of choice for BTRE, nor is there any protocol to guide the use of these medications at every step of disease progression. Treatment strategies aimed at the tumor, that is surgical procedures, radio‑ and chemotherapy appear to influence seizure control. Conversely, some ASMs have also shown antitumor properties. The present review summarizes and retrospectively analyzes the literature on the pathogenesis and management of BTRE to provide an updated comprehensive understanding. Furthermore, the challenges and opportunities for developing future therapies aimed at BTRE are discussed.

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