Dysembryoplastic neuroepithelial tumors located in the caudate nucleus area: report of four cases

Abstract

Objective and importance: Dysembryoplastic neuroepithelial tumors (DNTs) histologically resemble gliomas but behave as stable lesions. These tumors initially were considered to be located exclusively within the supratentorial cortex. The four reported cases demonstrate that DNTs may also arise in the area of the caudate nuclei. Moreover, the peculiar topography of these lesions, which suggests a derivation from the subependymal plate, is in accordance with the putative origin of DNTs from secondary germinal layers.

Clinical presentation: The patients experienced partial seizures (two patients), generalized seizures (one patient), or headaches (one patient). All patients were young (17-26 yr) at the onset of symptoms, and all had normal results from their neurological examinations.

Intervention: All lesions demonstrated a pseudocystic appearance on computed tomographic scans, were hypointense on T1-weighted magnetic resonance imaging scans, hyperintense on T2-weighted magnetic resonance imaging scans, and did not show contrast enhancement. The four tumors similarly lined the left or right caudate nuclei and expanded within the homolateral ventricle (three patients) or both lateral ventricles (one patient). In one patient, the tumor also involved the adjacent paraolfactory cortex.

Conclusion: In all patients, stereotactic biopsies helped to identify a specific glioneuronal element of DNTs. None of the tumors was operated on. Radiotherapy was performed in only one patient. A long pre- and/or postbiopsy imaging follow-up, which was available in two nontreated patients (3 yr and 16 yr), demonstrated the perfect stability of the lesion. The occurrence of DNTs in this peculiar location needs to be considered to avoid misidentification as "ordinary" gliomas and prevent useless aggressive treatment.