Skip to main content

Long-Term Epilepsy Associated Tumors

Imaging Appearance

  • Living reference work entry
  • First Online:
Clinical Neuroradiology
  • 1945 Accesses

Abstract

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.

This publication is endorsed by: European Society of Neuroradiology (www.esnr.org).

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

Access this chapter

Institutional subscriptions

Abbreviations

ANET:

Angiocentric neuroepithelial tumor = angiocentric glioma

DNET:

Dysembryoplastic neuroepithelial tumors

FCD:

Focal cortical dysplasia

LEAT:

Long-term epilepsy-associated tumor

MVNT:

Multinodular and vacuolating neuronal tumor

PGNT:

Papillary glioneuronal tumor

PXA:

Pleomorphic xanthoastrocytoma

References

  • 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.

    Article  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • Park SH, Won J, Kim SI, et al. Molecular testing of brain tumor. J Pathol Transl Med. 2017;51:205–23.

    Article  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • Urbach H, Mast H, Egger K, Mader I. Presurgical MR imaging in epilepsy. Clin Neuroradiol. 2015;25(Suppl 2):151–5.

    Article  Google Scholar 

  • Van Gompel JJ. Cortical ependymoma: an unusual epileptogenic lesion. J Neurosurg. 2011;114:1187.

    Article  Google Scholar 

Suggestions for Further Reading

  • 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.

    Article  Google Scholar 

  • Blümcke I, Wiestler OD. Gangliogliomas: an intriguing tumor entity associated with focal epilepsies. J Neuropathol Exp Neurol. 2002;61:575–84.

    Article  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • Kim DH, Suh YL. Pseudopapillary neurocytoma of temporal lobe with glial differentiation. Acta Neuropathol (Berl). 1997;94:187–91.

    Article  CAS  Google Scholar 

  • 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.

    Article  Google Scholar 

  • Majores M, Von Lehe M, Fassunke J, et al. Tumor recurrence and malignant progression of gangliogliomas. Cancer. 2008;113:3355–63.

    Article  Google Scholar 

  • Perkins OC. Gangliogliomas. Arch Pathol Lab Med. 1926;2:11–7.

    Google Scholar 

  • 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.

    CAS  PubMed  Google Scholar 

  • 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.

    Article  Google Scholar 

  • Sontowska I, Matyja E, Malejczyk J, Grajkowska W. Dysembryoplastic neuroepithelial tumour: insight into the pathology and pathogenesis. Folia Neuropathol. 2017;55:1–13.

    PubMed  Google Scholar 

  • Thom M, Blümcke I, Aronica E. Long-term epilepsy-associated tumors. Brain Pathol. 2012;22:350–79.

    Article  Google Scholar 

  • 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.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to H. Urbach .

Editor information

Editors and Affiliations

Section Editor information

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Urbach, H. (2019). Long-Term Epilepsy Associated Tumors. In: Barkhof, F., Jager, R., Thurnher, M., Rovira Cañellas, A. (eds) Clinical Neuroradiology. Springer, Cham. https://doi.org/10.1007/978-3-319-61423-6_52-2

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-61423-6_52-2

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-61423-6

  • Online ISBN: 978-3-319-61423-6

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics