Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update

Intraventricular dysembryoplastic neuroepithelial tumor in the temporal horn with Broad involvement of the ependyma

“…Reported intraventricular DNETs are often located at the vicinity of the third ventricle, sometimes associated with hydrocephalus. 5 Given the established correlation of DNETs with epilepsy, wright noninvasive diagnosis of DNETs would facilitate the clinical decision for surgical treatment of the associated epilepsy. 6 In this respect, knowing that atypical forms of DNETs could occur inside the ventricular system is of great value.…”

A Case of Dysembryoplastic Neuroepithelial Tumor in an Adolescent Male

“…Dysembryoplastic neuroepithelial tumors (DNETs) are benign mixed glioneuronal neoplasms that frequently occur in children and young adults [1], especially in the age group of 10-14 years, which is described to present the most significant peak; the incidence is higher in males than females [2]. They were described for the first time in 1998 by Damas-Duport [3] and are characterized histologically by the presence of oligodendrocyte-like cells [4].…”

Temporal lobe myxoid glioneuronal tumor, PDGFRA p.K385L‐mutant with DNA methylation confirmation

“…Histologically similar to d ysembryoplastic neuroepithelial tumor (DNT) or, less commonly, to rosette‐forming glioneuronal tumor, MGNT is currently defined by the presence of specific mutation in the platelet‐derived growth factor receptor α ( PDGFRA ) gene, namely, p.K385 mutation (changing K385 from a basic amino acid to a hydrophobic amino acid, usually L or I). MGNT was initially described in the septum pellucidum, septal nuclei, corpus callosum and lateral ventricle/periventricular white matter ( 2 , 3 , 4 ), but has recently been reported in the midbrain tectum by our group ( 5 ). We now add to the literature a second example of a non‐septum pellucidum location for this tumor in the temporal lobe, with both the mutation and confirmation by DNA methylation testing.…”