Gliome de bas grade avec altération de MYBL1 : observation d’une nouvelle entité tumorale pédiatrique

auteurs

• Appay Romain
• Tauziède-Espariat Arnault
• Silva Karen
• Fritih Radia
• Scavarda Didier
• Delteil Clémence
• Varlet Pascale
• Figarella‑branger Dominique

mots-clés

• Diffuse glioma
• Epilepsy
• Gliome diffus
• Isomorphe
• MYBL1
• MYBL1 Isomorphic
• Pediatric
• Pédiatrie
• Épilepsie

résumé

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.

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