%0 Journal Article
%A Aboubakr, Oumaima
%A Wefers, Annika K
%A Dangouloff-Ros, Volodia
%A Métais, Alice
%A Sievers, Philipp
%A Hasty, Lauren
%A Saffroy, Raphaël
%A Pierron, Gaelle
%A Guillemot, Delphine
%A Samoyeau, Thomas
%A Boddaert, Nathalie
%A Grill, Jacques
%A Beccaria, Kevin
%A Blauwblomme, Thomas
%A Varlet, Pascale
%A Tauziède-Espariat, Arnault
%T The malignant transformation of an atypical angiocentric glioma, MYB-altered.
%J Acta Neuropathologica Communications
%V 13
%N 1
%@ 2051-5960
%C London
%I Biomed Central
%M DKFZ-2025-01540
%P 161
%D 2025
%X According to the current World Health Organization classification of central nervous system tumors, the angiocentric glioma (AG) assigned a grade 1, characterized by recurrent MYB fusions. However, it also mentions that increased proliferative activity and other anaplastic features have been reported, but the clinical significance of such findings is unclear as an increased proliferative activity alone does not necessarily alter the benign behavior of AGs. Most cases with 'anaplasia' were mainly described before the molecular era. Herein, we report the case of a 3-year-old female with epilepsy symptomatic of a left temporo-parietal tumor. Histopathologically, the initial tumor displayed atypical AG morphology and a proliferation index of 1
%K Humans
%K Female
%K Glioma: genetics
%K Glioma: pathology
%K Glioma: diagnostic imaging
%K Brain Neoplasms: genetics
%K Brain Neoplasms: pathology
%K Brain Neoplasms: diagnostic imaging
%K Child, Preschool
%K Proto-Oncogene Proteins c-myb: genetics
%K Cell Transformation, Neoplastic: genetics
%K Cell Transformation, Neoplastic: pathology
%K Trans-Activators: genetics
%K Neoplasm Recurrence, Local: pathology
%K Neoplasm Recurrence, Local: genetics
%K Telomerase: genetics
%K Proto-Oncogene Proteins
%K Aggressive (Other)
%K Angiocentric glioma (Other)
%K DNA-methylation profiling (Other)
%K MYB (Other)
%K Proto-Oncogene Proteins c-myb (NLM Chemicals)
%K MYB protein, human (NLM Chemicals)
%K Trans-Activators (NLM Chemicals)
%K MYBL1 protein, human (NLM Chemicals)
%K Telomerase (NLM Chemicals)
%K Proto-Oncogene Proteins (NLM Chemicals)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:40702569
%R 10.1186/s40478-025-02070-4
%U https://inrepo02.dkfz.de/record/303121