State-of-the-Art Management of Children with Intractable Epilepsy and Brain Tumors

BACKGROUND: Epilepsy is a common and frequently debilitating manifestation of pediatric brain tumors, especially low-grade epilepsy-associated tumors (LEATs) such as gangliogliomas, DNETs, and PXAs. In many children, seizures are the presenting symptom and may evolve into intractable epilepsy, affecting neurocognitive development and quality of life. Understanding the relationship between tumor biology, epileptogenic networks, and clinical presentation is essential for guiding timely and effective management. SUMMARY: This review synthesizes current evidence and expert consensus on the integrated management of tumor-related epilepsy in children. We describe the anatomical, histopathological, and molecular correlates of epileptogenicity in pediatric brain tumors, with particular attention to the distinct clinical behavior of LEATs. Diagnostic strategies, including advanced imaging, neurophysiology, and invasive monitoring, are reviewed in the context of localizing the seizure onset zone while preserving function. The review outlines the indications and strategies for early surgical intervention, emphasizing that seizure control should be viewed as a primary therapeutic goal rather than a secondary outcome of tumor resection. Surgical approaches are discussed in relation to epilepsy duration, tumor location, and electroclinical concordance. For early, well-localized cases, lesionectomy may be sufficient, while longstanding epilepsy or tumors near eloquent cortex often require epilepsy-directed resections guided by intracranial monitoring. The role of preoperative ancillary tests (such as MEG, TMS, and neuropsychology) and intraoperative techniques (including ECoG and IONM) is highlighted for improving safety and precision. KEY MESSAGES: (i) Epilepsy is a significant contributor to morbidity in children with brain tumors, particularly LEATs. (ii) Multidisciplinary care is essential for seizure localization and for optimizing outcomes in children with tumor-related epilepsy. (iii) Early surgical intervention is associated with better seizure and cognitive outcomes. (iv) Delayed treatment increases the risk of secondary epileptogenesis and long-term developmental effects. (v) Individualized surgical planning should balance oncologic and epilepsy goals from the first clinical encounter. (vi) Ancillary diagnostic and intraoperative tools enhance safety and help achieve maximal functional preservation.