Clinicopathological features of dysembryoplastic neuroepithelial tumor: a case series

Abstract

Background Dysembryoplastic neuroepithelial tumors are rare benign supratentotrial epilepsy-associated glioneuronal tumors of children and young adults. Patients have a long history of seizures. Proper surgical resection achieves long term seizure control. We describe the clinicopathological features of dysembryoplastic neuroepithelial tumor cases reported in our practice and review the published literature. Methods All cases of Pakistani ethnicity were diagnosed between 2015 and 2021 were included. Slides were reviewed and clinicopathological features were recorded. Follow-up was obtained. Extensive literature review was conducted. Results Fourteen cases were reported. There were 12 males and 2 females. Age range was 9–45 years (mean 19 years). Majority were located in the temporal and frontal lobes. Duration of seizures prior to resection ranged from 2 months to 9 years with mean and median duration of 3.2 and 3 years, respectively. Histologically, all cases demonstrated a multinodular pattern, specific glioneuronal component, and floating neurons. Simple and complex forms comprised seven cases each. No significant nuclear atypia, mitotic activity, or necrosis was seen. Ki-67 proliferative index was very low. Cortical dysplasia was noted in adjacent glial tissue in four cases. Follow-up ranged from 20 to 94 months. Seizures continued following resection in all but one case but were reduced in frequency and intensity. In one case, seizures stopped completely following surgery. Conclusion Clinicopathological features were similar to those in published literature. However, a marked male predominance was noted in our series. Seizures continued following resection in all but one case but were reduced in frequency and intensity. This series will help raise awareness among clinicians and pathologists in our part of the world about this seizure-associated tumor of children and young adults.

Pilocytic astrocytoma: The paradigmatic entity in low‑grade gliomas (Review)

“…Additionally, PXA is most commonly found in the cerebral hemisphere and the leptomeninges; it occasionally occurs in the spinal cord, rarely evolving into a more malignant tumour ( 20 ). However, PA differs from dysembryoplastic neuroepithelial tumours by the absence of mucin-rich nodules and microcysts with floating neurons ( 2 , 21 ). Finally, the diffuse leptomeningeal glioneuronal tumour (DLGNT), a rare entity typically present in children, sometimes may present with focal areas of pilocytic features ( 2 ).…”