A rare, low-grade glioneuronal neoplasm characterized by a dinucleotide mutation at codon 385 of the PDGFR gene. It usually occurs in the septum pellucidum. It has also been described in the corpus callosum and periventricular white matter of the lateral ventricle. It has histologic features reminiscent of either dysembryoplastic neuroepithelial tumor or rosette-forming glioneuronal tumor. It is composed of oligodendrocyte-like cells in a prominent myxoid stroma. [from
NCI]