Patients with germline inactivation of the NF1 gene have a propensity to develop several tumors through acquired inactivation of the normal NF1 allele, and it has been estimated that 7% of individuals with NF1 develop GIST during their lifespan, which corresponds to a 34-fold higher risk than the average population [59,61]. Here, NF1 is linked to gastrointestinal stromal tumor.