Meningioma harboring IDH1 R132H mutation: a case report

Meningiomas are the most common primary central nervous system tumors. Monosomy of chromosome 22 is the most frequently reported genetic abnormality in meningioma and the occurrence of IDH1 mutations in meningioma is exceedingly rare. We report an uncommon case of IDH1 R132H-mutant meningioma. A 69-year-old man presented with two intracranial masses, one of which was identified as an IDH1 R132H-mutant meningioma by next-generation sequencing and further confirmed by pyrosequencing and immunohistochemistry. The rarity of this molecular finding and the potentially favorable prognosis observed in previously reported cases highlight the need for its recognition as a distinct molecular subset. Isocitrate dehydrogenase-targeted therapy may represent a future therapeutic option, pending validation through prospective studies and larger case series.