Mutation of the TERT telomerase reverse transcriptase (TERT) promoter is the second most common alteration in anaplastic PXA, after BRAF V600E mutation and CDKN2A homozygous deletion, and these genetic alterations could be related to anaplastic progression from PXA [2]. The gene discussed is TERT; the disease is pleomorphic xanthoastrocytoma.