IDH mutations are present in the vast majority of gliomas (Yan et al. 2009) and in approximately 17% of acute myeloid leukemia (Rakheja et al. 2012), though NSCLC with mutated IDH are seldom with an occurrence of 0.5–1.1% and only few cases are described yet (Rodriguez et al. 2020; Sequist et al. 2011; Toth et al. 2018). Here, IDH1 is linked to central nervous system cancer.