A rare cerebellar vermis high-grade neuroepithelial tumor

Neuroepithelial tumors, formerly known as primitive neuroectodermal tumors of the central nervous system, are reclassified under embryonal tumors in the 2016 WHO Classification of Tumors of the Central Nervous System. The tumor has two known genetic alterations: HGNET-MN1 and HGNET-BCOR. Previously, radiological features of the tumor have been reported as large, intra-axial lesions in the cerebral or cerebellar hemisphere, which presents mild adjacent edema. Here, we report the first case of high-grade neuroepithelial tumor not elsewhere classified (HGNET-NEC) arising from the cerebellar vermis, demonstrating good outcomes in clinical follow-up when compared with previously known types.