Skip to main content

Advertisement

SpringerLink
Log in

Dysembryoplastic neuroepithelial tumors and gangliogliomas: clinical results of 52 patients

  • Clinical Article
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Abstract

Purpose

Dysembryoplastic neuroepithelial tumors (DNET) and gangliogliomas (GG) are generally associated with epilepsy in young patients. Presurgical work-up and postsurgical results vary from center to center. Seizures are commonly focal with secondary generalization, and surgical treatment is often effective.

Methods

Twenty-eight patients with DNET and 24 patients with GG were eligible for this retrospective study. The authors present clinical, radiological, and pathological characteristics and seizure outcome of 52 patients histopathologically diagnosed with either a DNET or a GG.

Results

Characteristically the majority of the tumors showed hypointensity on T1-weighted images and increased signal intensity on both T2-weighted and FLAIR images. At the last follow-up (mean 54.3 months), overall favorable seizure outcome was 94.2% (n = 49). Twenty-six (92.8%) patients with DNET and 21 (87.5%) patients with GG were seizure free. Complete drug withdrawal was achieved in 26 (50%) patients. Shorter duration of epilepsy (p = 0.02), absence of status epilepticus (p = 0.01), absence of edema on MRI (p = 0.03), absence of seizure within the first month of surgery (p = 0.002), and total resection (p = 0.00001) were associated with favorable outcome with respect to seizure.

Conclusions

Our results indicate that a prompt diagnosis and total resection with additional adjacent structures or cortices when feasible are associated with a high epilepsy cure rate. Not only children but also adults benefit from surgical treatment. Although radiological features of DNETs or GGs are helpful, no definitive differences were found between the two types of tumors. Thus, clinical, radiological, and histopathological findings have to be considered together.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Awad IA, Rosenfeld J, Ahl J, Hahn JF, Luders H (1991) Intracrable epilepsy and structural lesions of the brain: mapping, resection strategies, and seizure outcome. Epilepsia 32:179–186

    Article  CAS  PubMed  Google Scholar 

  2. Shamji MF, Fric-Shamji EC, Benoit BG (2009) Brain tumours and epilepsy: pathophysiology of peritumoural changes. Neurosurg Rev 32:275–285

    Article  PubMed  Google Scholar 

  3. Daumas-Duport C, Pietsch T, Hawkins C, Shankar SK (2007) Dysembryoplastic neuroepithelial tumour. In: Louis DN, Oghaki H, Wiesttler D, Cavenee WK (eds) WHO classification of tumours of the central nervous system. WHO Press, Geneva, pp 99–102

    Google Scholar 

  4. Kleihues P, Burger PC, Scheithauer BW (1993) The new WHO classification of brain tumours. Brain Pathol 3:255–268

    Article  CAS  PubMed  Google Scholar 

  5. Prayson RA, Estes ML, Morris HH (1993) Coexistence of neoplasia and cortical dysplasia in patients presenting with seizures. Epilepsia 34:609–615

    Article  CAS  PubMed  Google Scholar 

  6. Perkins OC (1926) Ganglioglioma. Arch Pathol Lab Med 2:11–17

    Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  8. Haddad SF, Moore SA, Menezes AH, VanGildere JC (1992) Ganglioglioma: 13 years of experience. Neurosurgery 31:171–178

    Article  CAS  PubMed  Google Scholar 

  9. Aronica E, Leenstra S, van Veelen CWM, van Rijen PC, Hulsebos TJ, Tersmette AC, Yankaya B, Troost D (2001) Glioneuronal tumours and medically intractable epilepsy: a clinical study with long-term follow-up of seizure outcome after surgery. Epilepsy Res 43:179–191

    Article  CAS  PubMed  Google Scholar 

  10. Blumke I, Wiestler OD (2002) Gangliogliomas: an intriguing tumour entity associated with focal epilepsies. J Neuropathol Exp Neurol 61:575–584

    Google Scholar 

  11. Rushing EJ, Thompson LD, Mena H (2003) Malignant transformation of a dysembryoplastic neuroepithelial tumour after radiation and chemotherapy. Ann Diagn Pathol 7:240–244

    Article  PubMed  Google Scholar 

  12. Russell DS, Rubinstein LJ (1962) Ganglioglioma: a case with a long history and malignant evolution. J Neuropathol 21:185–193

    Article  CAS  Google Scholar 

  13. Sharma MC, Jain D, Gupta A, Sarkar C, Suri V, Garg A, Gaikwad SB, Chandra PS (2009) Dysembryoplastic neuroepithelial tumour: a clinicopathological study of 32 cases. Neurosurg Rev 32:161–170

    Article  PubMed  Google Scholar 

  14. Adachi Y, Yagishita A (2008) Gangliogliomas: characteristics imaging findings and role in the temporal lobe epilepsy. Neuroradiology 50:829–834

    Article  CAS  PubMed  Google Scholar 

  15. Bilginer B, Yalnizoglu D, Soylemezoglu F, Turanli G, Cila A, Topcu M, Akalan N (2009) Surgery for epilepsy in children with dysembryoplastic neuroepithelial tumour: clinical spectrum, seizure outcome, neuroradiology, and pathology. Childs Nerv Syst 25:485–491

    Article  PubMed  Google Scholar 

  16. Chan CH, Bittar RG, Davis GA, Kalnins RM, Fabinyi GCA (2006) Long-term seizure outcome following surgery for dysembryoplastic neuroepithelial tumour. J Neurosurg 104:62–69

    Article  PubMed  Google Scholar 

  17. Lee DY, Chung CK, Hwang YS, Choe G, Chi JG, Kim HJ, Cho BK (2000) Dysembryoplastic neuroepithelial tumours: radiological findings (including PET, SPECT, and MRS) and surgical strategy. J Neurooncol 47:167–174

    Article  CAS  PubMed  Google Scholar 

  18. Minkin K, Kelin O, Mancini J, Lena G (2008) Surgical strategies and seizure control in pediatric patients with dysembryoplastic neuroepithelial tumours: a single-institution experience. J Neurosurg Pediatrics 1:206–210

    Article  Google Scholar 

  19. Morris HH, Matkovic Z, Estes ML, Prayson RA, Comair YG, Turnbull J, Najm I, Kotagal P, Wyllie E (1998) Ganglioglioma and intractable epilepsy: clinical and neurophysiologic features and predictors of outcome after surgery. Epilepsia 39:307–313

    Article  CAS  PubMed  Google Scholar 

  20. Park YS, Kim DS, Shim KW, Kim JH, Choi JU (2008) Factors contributing to resectibility and seizure outcomes in 44 patients with ganglioglioma. Clin Neurol Neurosurg 110:667–673

    Article  PubMed  Google Scholar 

  21. Zentner J, Wolf HK, Ostertun B, Hufnagel A, Campos MG, Solymosi L, Schramm J (1994) Gangliogliomas: clinical, radiological, and histopathological findings in 51 patients. J Neurol Neurosurg Psychiatry 57:1497–1502

    Article  CAS  PubMed  Google Scholar 

  22. Engel J Jr, Van Ness PC, Rasmussen T (1993) Outcome with respect to epileptic seizures. In: Engel J Jr (ed) Surgical treatment of the epilepsies. Raven Press, New York, pp 609–621

    Google Scholar 

  23. Brainer-Lima PT, Brainer-Lima AM, Azevedo-Filho HR (2006) Ganglioglioma: comparison with other low-grade brain tumours. Arq Neuropsiquiatr 64:613–618

    PubMed  Google Scholar 

  24. Burneo JG, Tellez-Zenteno J, Steven DA, Niaz N, Hader W, Pillay N, Wiebe S (2008) Adult-onset epilepsy associated with dysembryoplastic neuroepithelial tumours. Seizure 17:498–504

    Article  CAS  PubMed  Google Scholar 

  25. Cosson SR, Varlet P, Beuvon F, Daumos-Duport C, Devaux B, Chassoux B, Fredy D, Meder JF (2001) Dysembryoplastic neuroepithelial tumours: CT, MR findings and imaging follow-up: a study of 53 cases. J Neuroradiol 28:230–240

    Google Scholar 

  26. Giulioni M, Gardella E, Rubboli G, Roncafoli F, Zucchelli M, Bernardi B, Tassinari CA, Calbucci F (2006) Lesionectomy in epileptogenic gangliogliomas: seizure outcome and surgical results. J Clin Neurosci 13:529–535

    Article  PubMed  Google Scholar 

  27. Im SH, Chung CK, Cho BK, Lee SK (2002) Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. J Neurooncol 57:59–66

    Article  PubMed  Google Scholar 

  28. Luyken C, Blumke I, Fimmers R, Urbach H, Wiestler OD, Schramm J (2004) Supratentorial gangliogliomas: histopathologic grading and tumour recurrence in 184 patients with a median follow-up of 8 years. Cancer 101:146–155

    Article  PubMed  Google Scholar 

  29. Majores M, von Lehe M, Fassunke J, Schramm J, Becker AJ, Simon M (2008) Tumour recurrence and malignant progression of gangliogliomas. Cancer 113:3355–3363

    Article  PubMed  Google Scholar 

  30. Takahashi A, Hong SC, Seo DW, Hong SB, Lee M, Suh YL (2005) Frequent association of cortical dysplasia in dysembryoplastic neuroepithelial tumour treated by epilepsy surgery. Surg Neurol 64:419–427

    Article  PubMed  Google Scholar 

  31. Villarejo F (2008) Resective neocortical techniques in children. In: Luders HO (ed) Epilepsy surgery, 1st edn. Informa, London, pp 1110–1120

    Google Scholar 

  32. Dean AC, Solomon G, Harden C, Papakostas G, Labar DR (1997) Left hemispheric dominance of epileptiform discharges. Epilepsia 38:503–505

    Article  CAS  PubMed  Google Scholar 

  33. Tampieri D, Moumdjian R, Melanson D, Ethier R (1991) Intracerebral gangliogliomas in patients with partial complex seizures: CT and MR imaging findings. AJNR Am J Neuroradiol 12:749–755

    CAS  PubMed  Google Scholar 

  34. Alexiou GA, Varela M, Sfakianos G, Prodromou N (2009) Benign lesions accompanied by intractable epilepsy in children. J Child Neurol 24:697–700

    Article  PubMed  Google Scholar 

  35. Bauer R, Dobesberger J, Unterhofer C, Interberger I, Walser G, Bauer G, Trinka E, Ortler M (2007) Outcome of adult patients with temporal lobe tumours and medically refractory focal epilepsy. Acta Neurochir (Wien) 149:1211–1217

    Article  CAS  Google Scholar 

  36. Ruban D, Byrne RW, Kanner A, Smith M, Cochran EJ, Roh D, Whisler WW (2009) Chronic epilepsy associated with temporal tumours: long-term surgical outcome. Neurosurg Focus 27(2):E6

    Article  PubMed  Google Scholar 

  37. Sandberg DI, Ragheb J, Dunoyer C, Bhatia S, Olavarria G, Morrison G (2005) Surgical outcomes and seizure control rates after resection of dysembryoplastic neuroepithelial tumours. Neurosurg Focus 18(6A):E5

    PubMed  Google Scholar 

Download references

Acknowledgments

We greatly appreciate the valuable help of Ozlem Uyanik, M.D. and Gulsum Akdeniz, PhD. in collecting the data from the archive and Buge Oz, M.D. for histopathological diagnosis.

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey

    Fatma Ozlen, Ziya Asan, Taner Tanriverdi, Emin Ozyurt & Mustafa Uzan

  2. Department of Neurology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey

    Aysegul Gunduz, Cigdem Ozkara, Naz Yeni & Cengiz Yalcinkaya

Authors
  1. Fatma Ozlen
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Aysegul Gunduz
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ziya Asan
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Taner Tanriverdi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Cigdem Ozkara
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Naz Yeni
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Cengiz Yalcinkaya
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Emin Ozyurt
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Mustafa Uzan
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mustafa Uzan.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ozlen, F., Gunduz, A., Asan, Z. et al. Dysembryoplastic neuroepithelial tumors and gangliogliomas: clinical results of 52 patients. Acta Neurochir 152, 1661–1671 (2010). https://doi.org/10.1007/s00701-010-0696-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00701-010-0696-4

Keywords

Search

Navigation