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Case Eight - Dysembryoplastic Neuroepithelial Tumor

Fig.1 Fig.2 Fig.3

Fig.4 Fig.5 Fig.6

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Clinical History: 13- year-old female with new onset of seizures.

Findings: T1-weighted coronal Turboflash images (Figs 1 and 2) and T1-weighted axial spin-echo images (Fig 5) demonstrate a well-defined, lobulated, low-signal mass involving the cortex and subcortical white matter of the left temporal lobe. The mass does not enhance following Gadolinium administration (Figs 3 and 6). The lesion is high in signal on the T2-weighted coronal images (Fig 4). It exhibits mild mass effect but there is no significant associated vasogenic edema.

Diagnosis: Dysembryoplastic Neuroepithelial Tumor

Discussion: Dysembryoplastic Neuroepithelial Tumor (DNT) is a recently recognized, benign tumor associated with medically intractable, partial complex seizures. Mean age of onset of symptoms is nine years (range 1-19 years). All reported DNT's have been supratentorial, most often involving the temporal lobe (approximately 2/3) followed in frequency by the frontal lobe (1/3). The tumors are primarily cortical in location, although they may extend to involve the subcortical white matter. On CT scans, DNT's are well-defined, low-attenuation lesions which may be mistaken for cysts. The tumors tend to be low signal on T1- weighted MR images and high signal on T2-weighted images, i.e., similar to CSF, but on proton-density images, they are slightly higher in signal than CSF, allowing them to be differentiated from simple cysts. Less than 25% calcify or enhance. There is associated calvarial remodeling in approximately 1/3 of cases.

Differential diagnoses include ganglioglioma and low-grade astrocytoma. A ganglioglioma, however, is more likely to be located within the white matter. Calvarial remodeling, if present, is a helpful differentiating feature in that it would be unlikely for either a ganglioglioma or a low-grade astrocytoma to cause such changes.

The prognosis for patients with DNT is excellent. 70-81% of patients are seizure-free following surgery, and the tumor does not recur, even in cases where resection is considered incomplete.

References:
Daumas-Duport C et al: Dysembryoplastic Neuroepithelial Tumor:
A Surgically Curable Tumor of Young Patients With Intractable
Partial Seizures. Neurosurgery 23:545-556, 1988.

Kirkpatrick PJ, et al: Control of Temporal Lobe Epilepsy
Following en bloc Resection of Low-grade Tumors. J. Neurosurg
78:19-25, 1993.

Koeller KK, Dillon WP: Dysembryoplastic Neuroepithelial Tumors:
MR Appearance. AJNR 13:1319-1325, Sept/Oct 1992.

Morris HH, et al: Chronic Intractable Epilepsy as the Only
Symptom of Primary Brain Tumor. Epilepsia 34:1038-1043, 1993.

Taratuto AL, et al: Dysembryoplastic Neuroepithelial Tumor:
Morphological Immunocytochemical, and Deoxyribonucleic Acid
Analyses in a Pediatric Series. Neurosurgery 36:474-481, 1995.

Toshiro K, et al: Radiologic Appearance of the Dysembryoplastic
Neuroepithelial Tumor. Radiology 197:233-238, 1995.

Vali AM, Clarke MA, Kelsey A: Dysembryoplastic Neuroepithelial
Tumour as a Potentially Treatable Cause of Intractable Epilepsy
in Children. Clinical Radiology 47:255-258, 1993.

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Submitted by:
Barbara Bangert, M.D.
Neuroradiology