Loss of NF1 results in increased Ras signaling effector and downstream pathways such as MAPK and mTOR signaling, which can cause unchecked cell proliferation.5 Benign and malignant tumor development is connected to a “second hit” somatic mutation in the NF1 gene, which results from a loss of heterozygosity (LOH) and additional mutations.6,7 Generally, tumors are initiated by neurofibromin-deficient (NF1−/−) Schwann cells, and driven by NF1 haploinsufficient microenvironments.1,2. Here, NF1 is linked to cancer.