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DOI: 10.1055/s-0043-1777730
Long-term epilepsy associated-tumors (LEATs): what is new?
Tumores associados a epilepsia de longa duração: o que há de novo?
Abstract
Long-term epilepsy-associated tumors (LEATs) include a series of neoplasms that commonly occur in children, adolescents, or young adults, have an astrocytic or glioneuronal lineage, are histologically benign (WHO grade1) with a neocortical localization predominantly situated in the temporal lobes. Clinically, chronic refractory epilepsy is usually the unique symptom. Gangliogliomas (GG) and dysembryoplastic neuroepithelial tumors (DNT) are the most common representative entities besides pilocytic astrocytomas (PA) and angiocentric gliomas (AG). Recent molecular studies have defined new clinicopathological entities, which are recognized by the WHO 2021 classification of brain tumors. Some of them such as diffuse astrocytoma MIB or MYBL1 altered, polymorphous low-grade neuroepithelial tumor of the young (PLNTY), and multilocular and vacuolating neuronal tumor (MVNT) are currently considered LEATs. The relationship between LEATs and epilepsy is still a matter of debate, and there is a general agreement about the beneficial effects of an early neurosurgical intervention on the clinical outcome.
Resumo
Tumores associados a epilepsia de longa duração constituem uma série de neoplasias asatrocitárias ou glioneuronais que comumente incidem em crianças, adolescentes e jovens adultos e que são histologicamente benignos (OMS grau 1), de localização neocortical e predominantemente situados nos lobos temporais. Clinicamente, a epilepsia crônica refratária é, de modo geral, o único sintoma. Gangliogliomas (GG) e tumores neuroepiteliais disembrioplásticos (DNT) são as entidades mais representativas associadas a astrocitomas pilocíticos (AP) e gliomas angiocêntricos (GA). Estudos moleculares recentes permitiram a definição de novas entidades clínico-patológicas reconhecidas pela classificação de tumores cerebrais da OMS 2021. Algumas delas, como o astrocitoma difuso MIB ou MIBL1 alterados, o tumor neuroepitelial polimorfo do jovem (PLNTY) e o tumor neuronal multilocular e vacuolizado (MVNT) são atualmente considerados tumores associados a epilepsia de longa duração. A relação entre este grupo de tumores e epilepsia é ainda debatida e há um consenso geral sobre o benefício prognóstico de intervenção cirúrgica precoce.
Publication History
Received: 25 August 2023
Accepted: 17 November 2023
Article published online:
29 December 2023
© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)
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References
- 1 Blümcke I, Spreafico R, Haaker G. et al; EEBB Consortium. – Histopathological findings in brain tissue obtained during epilepsy surgery. N Engl J Med 2017; 377 (17) 1648-1656
- 2 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 (06) 822-830
- 3 Thom M, Blümcke I, Aronica E. Long-term epilepsy-associated tumors. Brain Pathol 2012; 22 (03) 350-379
- 4 Xie M, Wang X, Duan Z, Luan G. Low-grade epilepsy-associated neuroepithelial tumors: Tumor spectrum and diagnosis based on genetic alterations. Front Neurosci 2023; 16: 1071314
- 5 Blümcke I, Aronica E, Urbach H, Alexopoulos A, Gonzalez-Martinez JA. A neuropathology-based approach to epilepsy surgery in brain tumors and proposal for a new terminology use for long-term epilepsy-associated brain tumors. Acta Neuropathol 2014; 128 (01) 39-54
- 6 Mann C, Conradi N, Neuhaus E. et al. – Early epilepsy surgery in benign cerebral tumors: avoid your “low-grade” becoming a long-term” epilepsy-associated tumor. J Clin Med 2022; 11 (19) 5892
- 7 Xie M, Wang X, Qiao J. et al. The clinical and pathological features of low-grade epilepsy-associated glioneuronal tumors. Sci Rep 2022; 12 (01) 18163
- 8 Blümcke I, Aronica E, Becker A. et al. Low-grade epilepsy-associated neuroepithelial tumours - the 2016 WHO classification. Nat Rev Neurol 2016; 12 (12) 732-740
- 9 Central Nervous System Tumours. WHO Classification of Tumours. 5th ed.. Lyons (France): International Agency for Research on Cancer; 2021
- 10 Zanello M, Pages M, Tauziède-Espariat A. et al. – Clinical, Imaging, histopathological and molecular characterization of anaplastic ganglioglioma. J Neuropathol Exp Neurol 2016; 75 (10) 971-980
- 11 Solomon DA, Blümcke I, Capper D, Gupta K. Varlet P – Gangliopglioma. In: Central Nervous System Tumours: WHO Classification of Tumours. 5th ed.. Lyons (France): International Agency for Research on Cancer; 2021: 111-115
- 12 Zaky W, Patil SS, Park M. et al. Ganglioglioma in children and young adults: single institution experience and review of the literature. J Neurooncol 2018; 139 (03) 739-747
- 13 Slegers RJ, Blumcke I. Low-grade developmental and epilepsy associated brain tumors: a critical update 2020. Acta Neuropathol Commun 2020; 8 (01) 27
- 14 Thom M, Toma A, An S. et al. One hundred and one dysembryoplastic neuroepithelial tumors: an adult epilepsy series with immunohistochemical, molecular genetic, and clinical correlations and a review of the literature. J Neuropathol Exp Neurol 2011; 70 (10) 859-878
- 15 Pietsch T, Elison DW, Hirose T, Jacques TS, Schüller U. Varlet P – Dysembryoplastic neuroepithelial tumour. In: Central Nervous System Tumours: WHO Classification of Tumours. 5th ed.. Lyons (France): International Agency for Research on Cancer; 2021: 123-126
- 16 Blümcke I, Thom M, Aronica E. et al. The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission. Epilepsia 2011; 52 (01) 158-174
- 17 Rivera B, Gayden T, Carrot-Zhang J. et al. Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors. Acta Neuropathol 2016; 131 (06) 847-863
- 18 Rosemberg S, Fujiwara D. Epidemiology of pediatric tumors of the nervous system according to the WHO 2000 classification: a report of 1,195 cases from a single institution. Childs Nerv Syst 2005; 21 (11) 940-944
- 19 Tihan T, Figarella-Branger D, Giannini C. et al. – Pilocitic astrocytoma. In: Central Nervous System Tumours: WHO Classification of Tumours. 5th ed.. Lyons (France): International Agency for Research on Cancer; 2021: 83-89
- 20 Lellouch-Tubiana A, Boddaert N, Bourgeois M. et al. Angiocentric neuroepithelial tumor (ANET): a new epilepsy-related clinicopathological entity with distinctive MRI. Brain Pathol 2005; 15 (04) 281-286
- 21 Shakur SF, McGirt MJ, Johnson MW. et al. Angiocentric glioma: a case series. J Neurosurg Pediatr 2009; 3 (03) 197-202
- 22 Kurokawa R, Baba A, Emile P. et al. Neuroimaging features of angiocentric glioma: A case series and systematic review. J Neuroimaging 2022; 32 (03) 389-399
- 23 Wang H, Zhu J, Zhu P, Luo C. Angiocentric glioma: A case report and review of the literature. J Clin Neurosci 2021; 94: 179-185
- 24 Wefers AK, Stichel D, Schrimpf D. et al. Isomorphic diffuse glioma is a morphologically and molecularly distinct tumour entity with recurrent gene fusions of MYBL1 or MYB and a benign disease course. Acta Neuropathol 2020; 139 (01) 193-209
- 25 Hawkins CE, Blümcke I, Capper D. et al. - Diffuse astrocytoma, MYB or MYBL1-altered. In: Central Nervous System Tumours: WHO Classification of Tumours. 5th ed.. Lyons (France): International Agency for Research on Cancer; 2021: 56-58
- 26 Suh YY, Lee K, Shim YM. et al. MYB/MYBL1:QKI fusion-positive diffuse glioma. J Neuropathol Exp Neurol 2023; 82 (03) 250-260
- 27 Huse JT, Snuderl M, Jones DT. et al. Polymorphous low-grade neuroepithelial tumor of the young (PLNTY): an epileptogenic neoplasm with oligodendroglioma-like components, aberrant CD34 expression, and genetic alterations involving the MAP kinase pathway. Acta Neuropathol 2017; 133 (03) 417-429
- 28 Rosemblum MK, Blümcke I, Ellison DW. Huse JT – Polymorphous low-grade neuroepithelial tumor of the young. In: WHO Classification of Tumours. 5th ed.. Lyons (France): International Agency for Research on Cancer; 2021: 62-64
- 29 Huse JT, Edgar M, Halliday J, Mikolaenko I, Lavi E, Rosenblum MK. Multinodular and vacuolating neuronal tumors of the cerebrum: 10 cases of a distinctive seizure-associated lesion. Brain Pathol 2013; 23 (05) 515-524
- 30 Rosemblum MK, Giangaspero F, Giannini C, Huse JT, Komori T. Pekmezci M – Multinodular and vacuolating neuronal tumour. In: WHO Classification of Tumours. 5th ed.. Lyons (France): International Agency for Research on Cancer; 2021: 143-145
- 31 Blümcke I, Coras R, Wefers AK. et al. Review: Challenges in the histopathological classification of ganglioglioma and DNT: microscopic agreement studies and a preliminary genotype-phenotype analysis. Neuropathol Appl Neurobiol 2019; 45 (02) 95-107
- 32 Delev D, Daka K, Heynckes S. et al. Long-term epilepsy-associated tumors: transcriptional signatures reflect clinical course. Sci Rep 2020; 10 (01) 96
- 33 Pelliccia V, Deleo F, Gozzo F. et al. Early and late epilepsy surgery in focal epilepsies associated with long-term epilepsy-associated tumors. J Neurosurg 2017; 127 (05) 1147-1152
- 34 Faramand AM, Barnes N, Harrison S. et al. Seizure and cognitive outcomes after resection of glioneuronal tumors in children. Epilepsia 2018; 59 (01) 170-178
- 35 Mehrotra A, Singh S, Kanjilal S. et al. – Long-term epilepsy-associated tumors (LEATs): A single-center, retrospective series and review of literature on factors affecting the seizure outcome. World Neurosurg 2020; 144: e149-e155
- 36 Englot DJ, Berger MS, Barbaro NM, Chang EF. Factors associated with seizure freedom in the surgical resection of glioneuronal tumors. Epilepsia 2012; 53 (01) 51-57
- 37 Bale TA, Rosenblum MK. The 2021 WHO Classification of Tumors of the Central Nervous System: An update on pediatric low-grade gliomas and glioneuronal tumors. Brain Pathol 2022; 32 (04) e13060
- 38 Vranjac S, Rosemberg S. Pilocytic astrocytoma: a clinical study in a single Brazilian institution. JICNA 2016; 16: 114
- 39 van Breemen MS, Wilms EB, Vecht CJ. Epilepsy in patients with brain tumours: epidemiology, mechanisms, and management. Lancet Neurol 2007; 6 (05) 421-430
- 40 Pallud J, Capelle L, Huberfeld G. Tumoral epileptogenicity: how does it happen?. Epilepsia 2013; 54 (Suppl. 09) 30-34
- 41 Aronica E, Yankaya B, Jansen GH. et al. Ionotropic and metabotropic glutamate receptor protein expression in glioneuronal tumours from patients with intractable epilepsy. Neuropathol Appl Neurobiol 2001; 27 (03) 223-237
- 42 Koh HY, Kim SH, Jang J. et al. BRAF somatic mutation contributes to intrinsic epileptogenicity in pediatric brain tumors. Nat Med 2018; 24 (11) 1662-1668