A cortical dysembryoplastic neuroepithelial tumor initially occurring in the periventricular white matter

In this report, a rare case of dysembryoplastic neuroepithelial tumor (DNET) initially presented as a small white matter lesion with calcification adjacent to the lateral ventricle and extending to the frontal cortex after 7 years. This 1-year-old boy initially suffered from partial seizures. Initial CT revealed a small, low-density area surrounding a tiny calcified mass in the deep white matter of the left frontal lobe. Seven years later, his seizures had become intractable to antiepileptic agents, and MR imaging demonstrated a relatively large mass extending from the calcified lesion up to the adjacent cortical surface. He underwent surgery and the tumor was subtotally removed. Histological examination of the tumor verified it as a DNET consisting of clusters of small oligodendrocytes with floating neurons in the mucoid background. The pattern of the tumor progression in this case suggests that a DNET in the cortex originates from the subependymal germinal layer near the ventricle.