Nonetheless, these promising results have led to the current Children’s Oncology Group (COG) randomized studies ACNS1831 [NCT03871257] and ACNS1833 [NCT04166409], which are evaluating selumetinib versus standard carboplatin/vincristine chemotherapy in the upfront setting for patients with NF1-associated or non-NF1-associated low grade gliomas, respectively. Here, NF1 is linked to central nervous system cancer.