Dysembryoplastic neuroepithelial tumors: MR appearance

Abstract

Purpose: Dysembryoplastic neuroepithelial tumor (DNT) is a newly described, pathologically benign tumor arising within the supratentorial cortex and having a 100% association with partial complex seizures. We reviewed the MR appearance of the brains of six patients with DNT, without and with administration of gadolinium-DTPA, emphasizing the clinical and radiologic features.

Methods: The MR images in six patients (five male, one female) with pathologically proved DNT were reviewed. Five had partial complex seizures and one had partial simple seizures. Age at onset of partial seizures ranged from 2 to 19 years. Scans were obtained with conventional T1 and T2 weighting without and with the administration of gadolinium-DTPA. All patients had craniotomies for medically refractory seizures and pathologic examination of all specimens was available for review.

Results: MR demonstrated a focal cortical mass in all patients. Five were located in the temporal lobe and one was located within the occipital lobe. Two showed enhancement with gadolinium-DTPA. Calcification occurred in one lesion. Common features included very low signal intensity on T1-weighted images and high signal on T2-weighted images, similar to cerebrospinal fluid. Proton density images demonstrated slightly higher signal intensity in the lesion than cerebrospinal fluid. The margin of the tumor is well-circumscribed and may remodel the adjacent calvarium.

Conclusions: DNT, a newly described pathologic entity resulting in chronic, often medically, intractable seizures, has characteristic features on MR that allow it to be suggested in the differential diagnosis. These lesions may simulate benign cysts; however, increased signal intensity on proton density images should prompt further investigation in the proper clinical setting. Differentiation from low-grade astrocytomas and ganglioglioma is not possible by MR.