Abstract
CNS high-grade neuroepithelial tumor with MN1 alteration (CNS HGNET-MN1)
is a rare entity recently described. Thirteen CNS HGNET-MN1 patients
were identified using genome wide methylation arrays/RT-PCR across 7
institutions and were correlated with treatment and outcome. All
patients had surgery (GTR:9/STR:4) as initial management followed by
radiotherapy (focal:5/craniospinal:2/CyberKnife:1) and systemic
chemotherapy in 3 patients. Seven patients relapsed; 5 local and 2
metastatic despite complete resection and radiotherapy. Three patients
died due to their tumor relapse after 24 months despite upfront
radiotherapy. Treatment of CNS HGNET-MN1 remains a major challenge with
multiple failures, despite aggressive surgical resections and upfront
radiotherapy.