Kazuya Morita, Yoshio Nakashima, Nozomu Kurose, Atsuhiro Kawashima, Akihiro Nishiyama, Hironori Fujisawa
Myxoid glioneuronal tumor (MGT) is a newly delineated CNS WHO grade 1 neoplasm genetically characterized by PDGFRA p.K385L/I mutation. We describe a 14-year-old boy presenting with gelastic seizures caused by an intraventricular MGT. Intraoperative frozen section allowed minimal resection, preserving surrounding eloquent structures. Histology showed oligodendrocyte-like cells within myxoid stroma and scattered floating neurons, while molecular analysis revealed the canonical PDGFRA p.K385L mutation. At 13-month follow-up, the residual tumor remained stable without any further neurological dysfunction. This case highlights the importance of intraoperative diagnosis for surgical safety in MGT.