Dysembryoplastic neuroepithelial tumour mimicking high grade glioma

Introduction Dysembryoplastic neuroepithelial tumour (DNET) is a rare, low grade neuroglial tumour with distinct clinico-radiological features. In this study, we report a case of an unusual presentation of DNET. Case presentation A 25-year-old male presented with a new-onset generalized seizure. His interictal electroencephalogram (EEG) showed active right temporal epileptiform discharges with focal slowing. An MRI revealed an ill-defined cortical and subcortical mass with minor contrast enhancement and the presence of haemorrhagic foci. Features of mild mass effect were noted, raising suspicion for a Grade II/III glioma. The patient underwent surgical resection of the tumour. Pathological examination demonstrated a multinodular intracortical glioneuronal tumour with a low Ki-67 proliferative index. The features were diagnostic for DNET. Conclusion DNET is a rare, long-term epilepsy-associated tumour with distinct radiological and pathological features. This case showed an unusual clinical and radiological presentation of DNET, suggestive of a higher-grade lesion. Pathological diagnosis remains the gold standard for the diagnosis of DNET.