Diffuse Multifocal Bilateral Dysembryoplastic Neuroepithelial Tumor: A Very Unusual Case Report

Abstract

Dysembryoplastic neuroepithelial tumor (DNT) considered as a benign cortical glioneuronal neoplasm of children or young adults, typically present with drug-resistant focal epilepsy. DNTs are usually located in the temporal lobe but can found in any part of the supratentorial brain cortex. Multifocal DNTs have rarely reported. Here we present an eight years old boy with two years follow up, having a somewhat stable diffuse multinodular DNT of the most significant spatial extent that may have reported, involving cortical and subcortical left temporo-occipital lobe, bilateral basal ganglia and thalamus, presenting with headache, short stature, and behavioral disorder.

A Constellation of Atypical Findings in a Nine-Year-Old Child With Dysembryoplastic Neuroepithelial Tumors: A Case Report and Review of the Literature

“…Multifocality is also an atypical presentation for DNETs, as these tumors tend to be solitary. However, there have been rare case reports of multifocal DNETs [8,[12][13][14][15][16][17], which was first described by Leung et al in 1994 [13]. Furthermore, multifocal presentation has been reported in association with hereditary conditions such as Jacob's syndrome (47,XYY) [14] or neurofibromatosis type 1 (NF1) [18].…”

“…Contrast enhancement can be seen in roughly one-third of DNETs, and may appear nodular, ring-like, or heterogeneous, and often involve the periphery [ 2 ]. More rarely, lesions followed radiographically may demonstrate delayed contrast enhancement, which is thought to result from microvascular proliferation, ischemia, or hemorrhage instead of malignant transformation [ 8 ]. Jensen et al [ 9 ] also hypothesized that new enhancement could be a result of increased seizure activity in medial temporal lesions.…”