Dysembryoplastic Neuroepithelial Tumor and its Atypical Variant in Children –  Case Reports

Authors: D. Krahulík 1;  M. Vaverka 1;  L. Tučková 2;  M. Köcher 3
Authors‘ workplace: LF UP a FN Olomouc Neurochirurgická klinika 1;  LF UP a FN Olomouc Ústav klinické a molekulární patologie 2;  LF UP a FN Olomouc Radiologická klinika 3
Published in: Cesk Slov Neurol N 2013; 76/109(4): 492-496
Category: Case Report


In this short summary, the authors describe an atypical form of dysembryoplastic neuroepithelial tumor (DNET) in children that has a different clinical, radiological and cytological profile that the typical DNET. Epileptic seizures with good response to radical surgical resection are the main clinical feature of a typical DNET. These tumors are typically in frontal or temporal locations. We describe three patients who underwent surgery during the last eight months, including a girl with an atypical form of DNET presenting with atypical clinical and, mainly, cytogenetic findings.

Key words:
neuroepithelial tumors – atypical forms


1. Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, Laws ER jr, Vedrenne C. Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty- nine cases. Neurosurgery 1988; 23(5): 545– 556.

2. Kaye AH, Laws E jr (eds). Brain tumors: an encyclopedic approach. 2nd ed. New York: Churchill Livingstone 2001.

3. Campos AR, Clusmann H, von Lehe M, Niehusmann P, Becker AJ, Schramm J et al. Simple and complex dysembryoplastic neuroepithelial tumors (DNT) variants: clinical profile, MRI, and histopathology. Neuroradiology 2009; 51(7): 433– 443.

4. Ostertun B, Wolf HK, Campos MG, Matus C, Solymosi L, Elger CE et al. Dysembryoplastic neuroepithelial tumors: MR and CT evaluation. AJNR Am J Neuroradiol 1996; 17(3): 419– 430.

5. Urbach H. MRI of long‑term epilepsy‑associated tumors. Semin Ultrasound CT MR 2008; 29(1): 40– 46.

6. Raymond AA, Halpin SF, Alsanjari N, Cook MJ, Kitchen ND, Fish DR et al. Dysembryoplastic neuroepithelial tumor. Features in 16 patients. Brain 1994; 117(3): 461– 475.

7. Hamada H, Kurimoto M, Nagai S, Asahi T, HirashimaY, Endo S. A rare case of dysembryoplastic neuroepithelial tumour in occipital lobe presenting with only headache. J Clin Neurosci 2003; 10(2): 276– 278.

8. Bird- Lieberman G, Sethi K, Childs AM, Chumas P, Crimmins D, Ismail A et al. Diffuse hemispheric dysembryoplastic neuroepithelial tumor: a new radiological variant associated with early- onset severe epilepsy. J Neurosurg Pediatr 2011; 7(4): 416– 420.

9. Parmar HA, Hawkins C, Ozelame R, Chuang S, Rutka J,Blaser S. Fluid- attenuated inversion recovery ring sign as a marker of dyembryoplastic neuroepithelial tumors. J Comput Assist Tomogr 2007; 31(3): 348– 353.

10. Hamada H, Kurimoto M, Nagai S, Asahi T, Hirashima Y, Endo S. A rare case of dysembryoplastic neuroepithelial tumour in occipital lobe presenting with only headache. J Clin Neurosci 2003; 10(2): 276– 278.

11. Quarato PP, Gennaro GD, Manfredi M, Esposito V.Atypical Lennox- Gastaut syndrome successfully treated with removal of a parietal dysembryoplastic tumour. Seizure 2002; 11(5): 325– 329.

12. Drake J, Hoffman HJ, Kobayashi J, Hwang P, Becker LE. Surgical management of children with temporal lobe epilepsy and mass lesions. Neurosurgery 1987; 21(6): 792– 797.

13. Nolan MA, Sakuta R, Chuang N, Otsubo H, Rutka JT,Snead OC 3rd et al. Dysembryoplastic neuroepithelial tumors in childhood: long‑term outcome and prognostic features. Neurology 2004; 62(12): 2270– 2276.

14. Luyken C, Blümcke I, Fimmers R, Urbach H, Elger CE,Wiestler OD et al. The spectrum of long‑term epilepsy‑associated tumors: long‑term seizure and tumor outcome and neurosurgical aspects. Epilepsia 2003; 44(6): 822– 830.

Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 4

2013 Issue 4

Most read in this issue
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account