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

type de document

ART

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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