Low-grade developmental and epilepsy associated brain tumors: a critical update 2020

Acta Neuropathologica Communications
Rutger Juriaan Slegers, Ingmar Blümcke

Abstract

Brain tumors represent the second most frequent etiology in patients with focal seizure onset before 18 years of age and submitted to epilepsy surgery. Hence, this category of brain tumors, herein defined as low-grade, developmental, epilepsy-associated brain tumors (LEAT) is different from those frequently encountered in adults as (A): 77% of LEAT occur in the temporal lobe; (B): the vast majority of LEAT are of low malignancy and classified as WHO I°; (C): LEAT are often composed of mixed glial and neuronal cell components and present with variable growth patterns including small cysts or nodules; (D): LEAT do not share common gene driving mutations, such as IDH1 or 1p/19q co-deletions. Characteristic entities comprise the ganglioglioma (GG), the dysembryoplastic neuroepithelial tumor (DNT), the angiocentric glioma (AG), the isomorphic diffuse glioma (IDG) and the papillary glio-neuronal tumor (PGNT), representing 73.2% of 1680 tumors collected in a large German series of 6747 patients submitted to epilepsy surgery. In the realm of exciting discoveries of genetic drivers of brain tumors new genes have been also reported for LEAT. BRAF V600E mutations were linked to GG with CD34 expression, FGFR1 mutations to DNT, MYB alterati...Continue Reading

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Citations

Nov 25, 2020·Neurosurgery·Diana L Thomas, Christopher R Pierson
Dec 20, 2020·Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova·V S KhalilovN N Zavadenko
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Jul 21, 2021·Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova·V S KhalilovN N Zavadenko

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Methods Mentioned

BETA
methylation profiling

Software Mentioned

LEAT

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