Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype (pHGG H3/IDH WT) represents a heterogeneous group of tumors, which is characterized by histological high-grade features, absence of histone H3 and IDH mutations, and aggressive biological behavior (CNS WHO grade 4) (WHO Classification of Tumours Editorial Board, 2021). Here, IDH1 is linked to central nervous system cancer.