A curious case of T2-FLAIR mismatch in H3K27M mutant glioma

Diffuse midline gliomas are a rare relatively new classification of primary central nervous system tumors which include astrocytomas, oligodendrogliomas, and glioblastomas. The T2-FLAIR mismatch sign is regarded as a highly specific imaging feature of IDH-mutant, 1p/19q non-codeleted astrocytomas. The case presented herein demonstrates this sign, however, in a non-IDH mutated diffuse midline glioma with a H3K27M mutation, a World Health Organization Grade IV neoplasm. Although preoperative diagnosis can provide important treatment and prognostic information, it is often quite difficult particularly in primary central nervous system tumors.