Long-Term Epilepsy Associated Tumors

Imaging Appearance
  • H. UrbachEmail author
Living reference work entry


Long-term epilepsy-associated tumors (LEATs) are the second common cause of drug-resistant epilepsy mostly occurring in young adults. Histologically, glioneuronal and glial tumors are distinguished. Gangliogliomas and dysembryoplastic neuroepithelial tumors (DNETs) account for the vast majority of glioneuronal tumors. They are located in the cortex or in the cortex and subcortical white matter; gangliogliomas most commonly in the mesial temporal lobe (“around the collateral sulcus”). MRI using contrast administration is the most important radiological technique. Both tumor types have typical imaging features in terms of location and imaging features, and clinical neuroradiology plays an important role in separating them from glial tumors. This separation is important since more than 70% of patients with drug-resistant epilepsy caused by gangliogliomas and DNETs will become seizure free following extended lesionectomy. Rarer glioneuronal tumors are angiocentric glioma (ANET) and papillary glioneuronal tumor (PGNT). Pleomorphic xanthoastrocytoma (PXA), isomorphic astrocytoma, and cortical ependymoma represent rarer epilepsy-associated glioma subtypes, while the association of glioneuronal tumors with neuropil islands and multinodular and vacuolating neuronal tumor (MVNT) of the cerebrum with epilepsy is less clear.


Epilepsy MRI Glioneuronal tumor Ganglioglioma DNET ANET PXA PGNT MVNT 

List of Abbreviations


Angiocentric neuroepithelial tumor = angiocentric glioma


Dysembryoplastic neuroepithelial tumors


Focal cortical dysplasia


Long-term epilepsy-associated tumor


Multinodular and vacuolating neuronal tumor


Papillary glioneuronal tumor


Pleomorphic xanthoastrocytoma


  1. Bien CG, Raabe AL, Schramm J, et al. Tendencies in characteristics of epilepsy patients undergoing presurgical evaluation and surgical treatment at one tertiary center from 1988-2009. J Neurol Neurosurg Psychiatry. 2013;84:54–61.CrossRefGoogle Scholar
  2. Blümcke I, Luyken C, Urbach H, et al. A new clinico-histopathological subtype of low-grade astrocytoma associated with long-term epilepsy and benign prognosis. Acta Neuropathol. 2004;107:381–8.CrossRefGoogle Scholar
  3. Blumcke I, Spreafico R, Haaker G, et al. Histopathological findings in brain tissue obtained from epilepsy surgery. N Engl J Med. 2017;377:1648–56.CrossRefGoogle Scholar
  4. Campos AR, Clusmann H, von Lehe M, et al. Simple and complex Dysembryoplastic Neuroepithelial Tumors (DNT): clinical profile, MRI and histopathology. Neuroradiology. 2009;51:433–43.CrossRefGoogle Scholar
  5. Heiland DH, Staszewski O, Hirsch M, et al. Malignant transformation of a Dysembryoplastic Neuroepithelial Tumor (DNET) characterized by genome-wide methylation analysis. J Neuropathol Exp Neurol. 2016;75:358–65.CrossRefGoogle Scholar
  6. Louis D, Perry A, Reifenberger G, et al. The 2016 World Health Organization classi cation of tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016;131:803–20.CrossRefGoogle Scholar
  7. Luyken C, Blümcke I, Fimmers R, et al. The spectrum of long-term epilepsy associated tumors: long-term seizure and tumor outcome and neurosurgical aspects. Epilepsia. 2003;44:822–30.CrossRefGoogle Scholar
  8. Nunes RH, Hsu CC, da Rocha AJ, et al. Multinodular and vacuolating neuronal tumor of the cerebrum: a new “Leave Me Alone” lesion with a characteristic imaging pattern. AJNR Am J Neuroradiol. 2017;38:1899–904.CrossRefGoogle Scholar
  9. Park SH, Won J, Kim SI, et al. Molecular testing of brain tumor. J Pathol Transl Med. 2017;51:205–23.CrossRefGoogle Scholar
  10. Schlamann A, von Bueren AO, Müller K. An individual patient data meta-analysis on characteristics and outcome of patients with papillary glioneuronal tumor, rosette glioneuronal tumor with neuropil-like islands and rosette forming glioneuronal tumor of the fourth ventricle. PLoS One. 2014;9:e101211.CrossRefGoogle Scholar
  11. Teo JG, Gultekin SH, Bilsky M, et al. A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including “rosetted”) islands: report of 4 cases. Am J Surg Pathol. 1999;23:502–10.CrossRefGoogle Scholar
  12. Urbach H, Mast H, Egger K, Mader I. Presurgical MR imaging in epilepsy. Clin Neuroradiol. 2015;25(Suppl 2):151–5.CrossRefGoogle Scholar
  13. Van Gompel JJ. Cortical ependymoma: an unusual epileptogenic lesion. J Neurosurg. 2011;114:1187.CrossRefGoogle Scholar

Suggestions for Further Reading

  1. Atri S, Sharma MC, Sarkar C, et al. Papillary glioneuronal tumour: a report of a rare case and review of literature. Childs Nerv Syst. 2007;23:349.CrossRefGoogle Scholar
  2. Blümcke I, Wiestler OD. Gangliogliomas: an intriguing tumor entity associated with focal epilepsies. J Neuropathol Exp Neurol. 2002;61:575–84.CrossRefGoogle Scholar
  3. Blumcke I, Aronica E, Urbach H, et al. A neuropathology-based approach to epilepsy surgery in brain tumors and proposal for a new terminology use for longterm epilepsy-associated brain tumors. Acta Neuropathol. 2014;128:39–54.CrossRefGoogle Scholar
  4. Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, et al. Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. Neurosurgery. 1988;23:545–56.CrossRefGoogle Scholar
  5. Furuta A, Takahashi H, Ikuta F, et al. Temporal lobe tumor demonstrating ganglioglioma and pleomorphic xanthoastrocytoma components. Case report. J Neurosurg. 1992;77:143–7.CrossRefGoogle Scholar
  6. Huse JT, Nafa K, Shukla N, et al. High frequency of IDH-1 mutation links glioneuronal tumors with neuropil-like islands to diffuse astrocytomas. Acta Neuropathol. 2011;122:367–9.CrossRefGoogle Scholar
  7. Huse JT, Edgar M, Halliday J, et al. Multinodular and vacuolating neuronal tumors of the cerebrum: 10 cases of a distinctive seizure-associated lesion. Brain Pathol. 2013;23:515–24.CrossRefGoogle Scholar
  8. Kepes JJ, Rubinstein LJ, Eng LF. Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young subjects with relatively favourable prognosis: a study of 12 cases. Cancer. 1979;44:1839–52.CrossRefGoogle Scholar
  9. Kim DH, Suh YL. Pseudopapillary neurocytoma of temporal lobe with glial differentiation. Acta Neuropathol (Berl). 1997;94:187–91.CrossRefGoogle Scholar
  10. Lellouch-Tubiana A, Boddaert N, Bourgeois C, et al. Angiocentric Neuroepithelial Tumor (ANET): a new epilepsy-related clinicopathological entity with distinctive MRI. Brain Pathol. 2005;15:281–6.CrossRefGoogle Scholar
  11. Majores M, Von Lehe M, Fassunke J, et al. Tumor recurrence and malignant progression of gangliogliomas. Cancer. 2008;113:3355–63.CrossRefGoogle Scholar
  12. Perkins OC. Gangliogliomas. Arch Pathol Lab Med. 1926;2:11–7.Google Scholar
  13. Saito T, Oki S, Mikami T, Kawamoto Y, Yamaguchi S, Kuwamoto K, et al. Supratentorial ectopic ependymoma: a case report. No Shinkei Geka. 1999;27:1139–44.PubMedGoogle Scholar
  14. Schramm J, Luyken C, Urbach H, et al. Evidence for a clinically distinct new subtype of grade II astrocytomas in patients with long-term epilepsy. Neurosurgery. 2004;55:340–58.CrossRefGoogle Scholar
  15. Sontowska I, Matyja E, Malejczyk J, Grajkowska W. Dysembryoplastic neuroepithelial tumour: insight into the pathology and pathogenesis. Folia Neuropathol. 2017;55:1–13.PubMedGoogle Scholar
  16. Thom M, Blümcke I, Aronica E. Long-term epilepsy-associated tumors. Brain Pathol. 2012;22:350–79.CrossRefGoogle Scholar
  17. Wang M, Tihan T, Rojiani AM, et al. Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. J Neuropathol Exp Neurol. 2005;64:875–81.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Neuroradiology, Faculty of MedicineMedical Center – University of FreiburgFreiburgGermany

Section editors and affiliations

  • N. Bargalló
    • 1
  1. 1.Magnetic Resonance Image Core Facility. Institut de Investigació Biomèdica August Pi I Sunyer (IDIBAPS)Image Diagnosis Center (CDIC). Hospital Clínic de BarcelonaBarcelonaSpain

Personalised recommendations