[Low grade glioma with MYBL1 alteration: Case report of an uncommon pediatric neoplasm]

Abstract

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.

Keywords: Diffuse glioma; Epilepsy; Gliome diffus; Isomorphe; MYBL1; MYBL1: Isomorphic; Pediatric; Pédiatrie; Épilepsie.