Papillary glioneuronal tumor presenting with recurrent epileptic seizures in a 6-year-old boy: A case report and comprehensive literature review

Background: Papillary glioneuronal tumor (PGNT) is a rare, World Health Organization (WHO) Grade I mixed glioneuronal neoplasm characterized by the presence of both neuronal and glial elements. It commonly presents with seizures or headaches and has a generally favorable prognosis after gross total resection. Here, we present an unusual case of PGNT with deep hemispheric extension involving the centrum semiovale, temporal lobe, and mesencephalon in a pediatric patient. Case Description: A 6-year-old male patient presented with a history of two partial epileptic seizures over the past 3 months. On admission, neurological examination was unremarkable. Personal and family histories were non-contributory. Initial cranial magnetic resonance imaging (MRI) revealed a heterogeneous T2-hyperintense, T1-hypointense, noncontrast-enhancing lobulated lesion extending from the left centrum semiovale to the temporal lobe and mesencephalon with minimal rightward midline shift. The patient underwent left frontoparietal craniotomy and microsurgical excision under neuronavigation. Given the tumor’s eloquent location, a safe subtotal resection was performed. Histopathological evaluation revealed a WHO Grade I PGNT with glial fibrillary acidic protein, Olig2, and synaptophysin positivity and a low Ki-67 proliferation index (2–3%). Postoperatively, the patient had no neurological deficits and was discharged on the 7 th day. A follow-up MRI is planned at 6 months. Conclusion: This case underscores the need to include PGNT in the differential diagnosis of pediatric patients presenting with new-onset focal seizures and MRI findings suggestive of a low-grade glioma. Surgical management is typically curative, resulting in excellent seizure control and long-term outcomes.