TY  - JOUR
AU  - Aboubakr, Oumaima
AU  - Wefers, Annika K
AU  - Dangouloff-Ros, Volodia
AU  - Métais, Alice
AU  - Sievers, Philipp
AU  - Hasty, Lauren
AU  - Saffroy, Raphaël
AU  - Pierron, Gaelle
AU  - Guillemot, Delphine
AU  - Samoyeau, Thomas
AU  - Boddaert, Nathalie
AU  - Grill, Jacques
AU  - Beccaria, Kevin
AU  - Blauwblomme, Thomas
AU  - Varlet, Pascale
AU  - Tauziède-Espariat, Arnault
TI  - The malignant transformation of an atypical angiocentric glioma, MYB-altered.
JO  - Acta Neuropathologica Communications
VL  - 13
IS  - 1
SN  - 2051-5960
CY  - London
PB  - Biomed Central
M1  - DKFZ-2025-01540
SP  - 161
PY  - 2025
AB  - 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
KW  - Humans
KW  - Female
KW  - Glioma: genetics
KW  - Glioma: pathology
KW  - Glioma: diagnostic imaging
KW  - Brain Neoplasms: genetics
KW  - Brain Neoplasms: pathology
KW  - Brain Neoplasms: diagnostic imaging
KW  - Child, Preschool
KW  - Proto-Oncogene Proteins c-myb: genetics
KW  - Cell Transformation, Neoplastic: genetics
KW  - Cell Transformation, Neoplastic: pathology
KW  - Trans-Activators: genetics
KW  - Neoplasm Recurrence, Local: pathology
KW  - Neoplasm Recurrence, Local: genetics
KW  - Telomerase: genetics
KW  - Proto-Oncogene Proteins
KW  - Aggressive (Other)
KW  - Angiocentric glioma (Other)
KW  - DNA-methylation profiling (Other)
KW  - MYB (Other)
KW  - Proto-Oncogene Proteins c-myb (NLM Chemicals)
KW  - MYB protein, human (NLM Chemicals)
KW  - Trans-Activators (NLM Chemicals)
KW  - MYBL1 protein, human (NLM Chemicals)
KW  - Telomerase (NLM Chemicals)
KW  - Proto-Oncogene Proteins (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:40702569
DO  - DOI:10.1186/s40478-025-02070-4
UR  - https://inrepo02.dkfz.de/record/303121
ER  -