Dysembryoplastic neuroepithelial tumors: nonspecific histological forms -- a study of 40 cases

Abstract

Objective: To demonstrate that DNTs include a large morphological spectrum of tumors that cannot be histologically distinguished from conventional categories of gliomas.

Methods: All tumors from patients who underwent epilepsy surgery in Sainte-Anne hospital (Paris) that histologically resembled gliomas and did not conform to current histological criteria for DNTs or gangliogliomas were entered in the study.

Results: According to the WHO histological classification, the 40 tumors resembled: pilocytic astrocytomas (4 cases), astrocytomas (16 cases), anaplastic astrocytoma (1 case), oligodendrogliomas (10 cases), oligo-astrocytomas (8 cases) or anaplastic oligo-astrocytomas (1 case). However foci of cortical dysplasia could be observed in 47% of the cases. Clinical presentation and imaging features were strikingly similar to that observed in typical DNTs. Although surgical removal was incomplete in 28% of the cases and none of the patients underwent chemo or radiotherapy, none of the tumors recurred (mean follow-up: 7 years). Moreover, serial preoperative imaging in 26 patients (mean follow-up: 4.5 years) demonstrated that these lesions were perfectly stable.

Conclusions: Whatever the histological appearance of a glial tumor, the diagnosis of DNT must be considered when all the following criteria are associated: (1) partial seizures, with or without secondary generalization, beginning before the age 20 years, (2) no neurological deficit or stable congenital deficit, (3) cortical topography of the lesion as better demonstrated by MRI and (4) no mass effect on imaging.