Abstract
The case of a 20 year old boy, with long-lasting focal symptomatic epilepsy and a poor control of his seizures, because of irregular adherence to the neurological treatment, is presented. Brain MRI showed a multi-cystic lesion, located mainly in the temporal lobe. A brain stereotaxic biopsy was performed, finding a specific neural gliosis in a mucin-like matrix. The diagnosis of dysembryoplastic neuroepithelial tumor was done. Patient had a good control of his seizures with valproic acid and carbamazepine. Patient improved his functional state; however his previous cognitive deficit remains unchanged. Surgical resection was not developed because the lesion is considered as very benign and because the control of his seizures.
The diagnosis of dysembryoplastic neuroepithelial tumor should be kept in mind in a patient with a long history of focal seizures; mainly when seizures onset begin before 20 years old, without motor or sensory neurological deficit, and when the MRI shows a cortical multi-cystic lesion, without mass effect, located in the temporal lobe.
References
Daumas-Duport C, Scheithauer BW, Chowkiewicz JP, Laws ER, Vedrenne C. Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Neurosurgery 1988; 23:545-556.
Cabiol J, Acebes JJ, Isamat F. Dysembryoplastic neuroepithelial tumor. Crit Rev Neurosurg 1999; 9:116-125.
Hirose T. Dysembryoplastic neuroepithelial tumor.
Neuropathol 1999; 19:233-237.
Raymond AA, Halpin SFS, Alsanjari N, Cook MJ, Kitchen ND, Fish DR et al. Dysembryoplastic neuroepithelial tumor. Features in 16 patients. Brain 1994; 117:461-475.
Kuchelmeister K, Demirel T, Schlörer E, Bergmann M, Gullotta F. Dysembryoplastic neuroepithelial tumor of the cerebellum. Acta Neuropathol (Berl) 1995; 89:385-390.
Raymond AA, Fish DR, Sisodiya N, Stevens JM, Shorvon SD. Abnormalities of gyration, heterotopias, tuberous sclerosis, focal cortical dysplasia, microdysgenesis, dysembryoplastic neuroepithelial tumor and dysgenesis of the archicortex in epilepsy. Clinical, EEG and neuroimaging features in 100 adult patients. Brain 1995;118:629-660.
Kuroiwa T, Bergey GK, Rothman MI, Zoarski GH, Wolf A, Zagardo MT et al. Radiological appearance of the dysembryoplastic neuroepithelial tumor. Radiology 1996;197: 233-238.
Stoodley MA, Ojeda VJ, Stokes BA, Lee MA, Thomas GW. Dysembryoplastic neuroepithelial tumour: the Western Australian experience. J Clin Neurosci 1997; 4: 155-160.
Daumas-Duport C. Dysembryoplastic neuroepithelial tumors. Brain Pathol 1993;3: 283-295.
Hirose T, Scheithauer BW. Mixed dysembryoplastic neuroepithelial tumor and ganglioglioma. Acta Neuropathol 1998;95: 649-654.
Daumas-Duport C, Varlet P, Bacha S, Beuvon F, Cervera-Pierot P, Chodkiewicz JP. Dysembryoplastic neuroepithelial tumors: nonspecific histological forms. A study of 40 cases. J Neurooncol 1999; 41:267-280.
Rushing EJ, Thompson LD, Mena H. Malignant Transformation of a Dysembryoplastic Neuroepithelial Tumor after Radiation and Chemotherapy. Ann Diagn Pathol 2003; 7: 240-244.
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