In only one published case, treatment beyond imatinib has been described in familial GIST; this case relates to a patient with familial GIST due to a germline KIT exon 13 variant p.Asn655Lys.10 This patient received sunitinib as second line of treatment and remained stable for a total of 15 months, which is consistent with response rates in sporadic GIST cases.10 Here, KIT is linked to gastrointestinal stromal tumor.