Objectives: Dysembryoplastic neuroepithelial tumors (DNTs) are usually located within the supratentorial cortex. We present a series of eight cases of DNTs including two cases with an extracortical location, one in the caudate nucleus, the other one expanded in the lateral ventricule, septum and fornix. An origin from secondary germinal layers, as previously suggested, can explain these extracortical locations.
Material and methods: Of the eight patients, seven had partial epileptic seizures and one intracranial hypertension. All patients underwent clinical examination, a computed tomographic (CT) scan, a magnetic resonance imaging (MRI) and a surgical removal of the lesion with histological examination.
Results: Clinical examination was normal except in the case with intracranial hypertension where a bilateral papillary oedema was found. In seven cases the CT scan showed a hypodense lesion of pseudocystic appearance. All lesions were hypointense on T1-weighted and hyperintense on T2-weighted MRI. Contrast enhancement was observed in two cases. The lesion was intracortical in six cases and extracortical in the remaining two: one in the head of the caudate nucleus and one in the trigonoseptal region. Histological examination identified an appearance of DNT with a specific glioneuronal element in six cases.
Conclusion: The diagnosis of DNT can be suspected before histological examination on radiological features, chiefly because the tumor is located in the supratentorial cortex. However, extracortical locations do exist, even if unusual. As DNTs are always benign, knowledge and accurate diagnosis of these atypical cases are mandatory in order to avoid useless and even deleterious additional treatments, such as radiotherapy.