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Diffuse gliomas with MYB or MYBL1 alterations are rare tumours mostly affecting children or young adults with long-term epilepsy. This category of glioma includes two morphological subtypes. The angiocentric subtype is characterized by an angiocentric pattern of growth and a frequent MYB:QKI fusion. The isomorphic subtype corresponds to a highly differentiated astrocytic glioma with low cellularity, low proliferation and no specific microscopic features. The diagnosis is based on the imaging, demonstrating a supratentorial tumor, associated with the confirmation of a MYB or MYBL1 rearrangement. Here, we report the case of a 7-year-old child who presented a right frontal brain lesion corresponding to an isomorphic diffuse glioma with MYBL1 alteration. Copyright © 2020 Elsevier Masson SAS. All rights reserved.

Citation

Romain Appay, Arnault Tauziède-Espariat, Karen Silva, Radia Fritih, Didier Scavarda, Clémence Delteil, Pascale Varlet, Dominique Figarella-Branger. Low grade glioma with MYBL1 alteration: Case report of an uncommon pediatric neoplasm]. Annales de pathologie. 2021 Feb;41(1):129-133

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PMID: 32732147

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