Indeed, in a minority of patients, GIST onset can be linked to Type 1 neurofibromatosis (NF1), characterized by a germline mutation of the NF1 gene, and Carney–Stratakis syndrome, marked by a germline pathogenic variant of one of the subunit genes of the SDH enzyme complex, linked to hypermethylation of the SDHC gene [20]. Here, SDHC is linked to neurofibromatosis type 1.